Progranulin: a new avenue towards the understanding and treatment of neurodegenerative disease

Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

Neumann, M., Sampathu, D. M., Kwong, L. K., Truax, A. C., Micsenyi, M. C., Chou, T. T., Bruce, J., Schuck, T., Grossman, M., Clark, C. M., et al. (2006) · Science · doi:10.1126/science.1134108 · Cited by 6,576

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

Arai, T., Hasegawa, M., Akiyama, H., Ikeda, K., Nonaka, T., Mori, H., Mann, D., Tsuchiya, K., Yoshida, M., Hashizume, Y., et al. (2006) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2006.10.093 · Cited by 2,732

Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17

Baker, M., Mackenzie, I. R., Pickering-Brown, S. M., Gass, J., Rademakers, R., Lindholm, C., Snowden, J., Adamson, J., Sadovnick, A. D., Rollinson, S., et al. (2006) · Nature · doi:10.1038/nature05016 · Cited by 2,058

The activation of Akt/PKB signaling pathway and cell survival

Song, G., Ouyang, G., & Bao, S. (2005) · Journal of Cellular and Molecular Medicine · doi:10.1111/j.1582-4934.2005.tb00337.x · Cited by 1,920

Lysosome biogenesis and lysosomal membrane proteins: trafficking meets function

Saftig, P., & Klumperman, J. (2009) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm2745 · Cited by 1,648

Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

Cruts, M., Gijselinck, I., van der Zee, J., Engelborghs, S., Wils, H., Pirici, D., Rademakers, R., Vandenberghe, R., Dermaut, B., Martin, J.-J., et al. (2006) · Nature · doi:10.1038/nature05017 · Cited by 1,512

The Transcription Factor TFEB Links mTORC1 Signaling to Transcriptional Control of Lysosome Homeostasis

Roczniak-Ferguson, A., Petit, C. S. V., Froehlich, F., Qian, S., Ky, J., Angarola, B. L., Walther, T. C., & Ferguson, S. M. (2012) · Science Signaling · doi:10.1126/scisignal.2002790 · Cited by 1,393

Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein

Watts, G. D. J., Wymer, J., Kovach, M. J., Mehta, S. G., Mumm, S., Darvish, D., Pestronk, A., Whyte, M. P., & Kimonis, V. E. (2004) · Nature Genetics · doi:10.1038/ng1332 · Cited by 1,391

Genetic compensation induced by deleterious mutations but not gene knockdowns

Rossi, A., Kontarakis, Z., Gerri, C., Nolte, H., Hölper, S., Krüger, M., & Stainier, D. Y. R. (2015) · Nature · doi:10.1038/nature14580 · Cited by 1,226

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

Deng, H.-X., Chen, W., Hong, S.-T., Boycott, K. M., Gorrie, G. H., Siddique, N., Yang, Y., Fecto, F., Shi, Y., Zhai, H., et al. (2011) · Nature · doi:10.1038/nature10353 · Cited by 1,174

A harmonized classification system for FTLD-TDP pathology

Mackenzie, I. R., Neumann, M., Baborie, A., Sampathu, D. M., du Plessis, D., Jaros, E., Perry, R. H., Trojanowski, J. Q., Mann, D. M. A., & Lee, V. M. ‐. (2011) · Acta Neuropathologica · doi:10.1007/s00401-011-0845-8 · Cited by 1,042

Sortilin is essential for proNGF-induced neuronal cell death

Nykjaer, A., Lee, R., Teng, K. K., Jansen, P., Madsen, P., Nielsen, M. S., Jacobsen, C., Kliemannel, M., Schwarz, E., Willnow, T. E., et al. (2004) · Nature · doi:10.1038/nature02319 · Cited by 962

Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia

Skibinski, G., Parkinson, N. J., Brown, J. M., Chakrabarti, L., Lloyd, S. L., Hummerich, H., Nielsen, J. E., Hodges, J. R., Spillantini, M. G., Thusgaard, T., et al. (2005) · Nature Genetics · doi:10.1038/ng1609 · Cited by 822

The Growth Factor Progranulin Binds to TNF Receptors and Is Therapeutic Against Inflammatory Arthritis in Mice

Tang, W., Lu, Y., Tian, Q.-Y., Zhang, Y., Guo, F.-J., Liu, G.-Y., Syed, N. M., Lai, Y., Lin, E. A., Kong, L., et al. (2011) · Science · doi:10.1126/science.1199214 · Cited by 761

Progranulin Deficiency Promotes Circuit-Specific Synaptic Pruning by Microglia via Complement Activation

Lui, H., Zhang, J., Makinson, S. R., Cahill, M. K., Kelley, K. W., Huang, H.-Y., Shang, Y., Oldham, M. C., Martens, L. H., Gao, F., et al. (2016) · Cell · doi:10.1016/j.cell.2016.04.001 · Cited by 729

The overlap of amyotrophic lateral sclerosis and frontotemporal dementia

Lomen, C. E., Anderson, T. T., & Miller, B. L. (2002) · Neurology · doi:10.1212/wnl.59.7.1077 · Cited by 699

Histone Deacetylase Inhibitors in Clinical Studies as Templates for New Anticancer Agents

Mottamal, M., Zheng, S., Huang, T. L., & Wang, G. (2015) · Molecules · doi:10.3390/molecules20033898 · Cited by 698

Conversion of Proepithelin to Epithelins

Zhu, J. K., Nathan, C., Jin, W., Sim, D., Ashcroft, G. S., Wahl, S. M., Lacomis, L., Erdjument‐Bromage, H., Tempst, P., Wright, C. D., et al. (2002) · Cell · doi:10.1016/s0092-8674(02)01141-8 · Cited by 634

Mutations in progranulin are a major cause of ubiquitin-positive frontotemporal lobar degeneration

Gass, J. M., Cannon, A. D., Mackenzie, I. R., Boeve, B. F., Baker, M. C., Adamson, J. L., Crook, R., Melquist, S., Kuntz, K., Petersen, R. C., et al. (2006) · Human Molecular Genetics · doi:10.1093/hmg/ddl241 · Cited by 587

Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions

Van Deerlin, V. M., Sleiman, P. M. A., Martinez-Lage, M., Chen-Plotkin, A., Wang, L.-S., Graff-Radford, N. R., Dickson, D. W., Rademakers, R., Boeve, B. F., Grossman, M., et al. (2010) · Nature Genetics · doi:10.1038/ng.536 · Cited by 579

The epidemiology of frontotemporal dementia

Onyike, C. U., & Diehl-Schmid, J. (2013) · International Review of Psychiatry · doi:10.3109/09540261.2013.776523 · Cited by 543

Presymptomatic cognitive and neuroanatomical changes in genetic frontotemporal dementia in the Genetic Frontotemporal dementia Initiative (GENFI) study: a cross-sectional analysis

Rohrer, J. D., Nicholas, J. M., Cash, D. M., van Swieten, J., Dopper, E., Jiskoot, L., van Minkelen, R., Rombouts, S. A., Cardoso, M. J., Clegg, S., et al. (2015) · The Lancet Neurology · doi:10.1016/s1474-4422(14)70324-2 · Cited by 538

Sortilin-Mediated Endocytosis Determines Levels of the Frontotemporal Dementia Protein, Progranulin

Hu, F., Padukkavidana, T., Vægter, C. B., Brady, O. A., Zheng, Y., Mackenzie, I. R., Feldman, H. H., Nykjaer, A., & Strittmatter, S. M. (2010) · Neuron · doi:10.1016/j.neuron.2010.09.034 · Cited by 536

Progranulin is a mediator of the wound response

He, Z., Ong, C. H. P., Halper, J., & Bateman, A. (2003) · Nature Medicine · doi:10.1038/nm816 · Cited by 490

Strikingly Different Clinicopathological Phenotypes Determined by Progranulin-Mutation Dosage

Smith, K. R., Damiano, J., Franceschetti, S., Carpenter, S., Canafoglia, L., Morbin, M., Rossi, G., Pareyson, D., Mole, S. E., Staropoli, J. F., et al. (2012) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2012.04.021 · Cited by 489

Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice

Yin, F., Banerjee, R., Thomas, B., Zhou, P., Qian, L., Jia, T., Ma, X., Ma, Y., Iadecola, C., Beal, M. F., et al. (2010) · Journal of Experimental Medicine · doi:10.1084/jem.20091568 · Cited by 480

Progranulin (granulin-epithelin precursor, PC-cell-derived growth factor, acrogranin) mediates tissue repair and tumorigenesis

He, Z., & Bateman, A. (2003) · Journal of Molecular Medicine · doi:10.1007/s00109-003-0474-3 · Cited by 477

Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival

Damme, P. V., Hoecke, A. V., Lambrechts, D., Vanacker, P., Bogaert, E., Swieten, J. C. V., Carmeliet, P., Bosch, L. V. D., & Robberecht, W. (2008) · The Journal of Cell Biology · doi:10.1083/jcb.200712039 · Cited by 421

Secretory leukocyte protease inhibitor mediates non-redundant functions necessary for normal wound healing

Ashcroft, G. S., Lei, K.-J., Jin, W., Longenecker, G., Kulkarni, A. B., Greenwell‐Wild, T., Hale‐Donze, H., McGrady, G., Song, X.-Y., & Wahl, S. M. (2000) · Nature Medicine · doi:10.1038/80489 · Cited by 416

Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies

Mackenzie, I. R., & Neumann, M. (2016) · Journal of Neurochemistry · doi:10.1111/jnc.13588 · Cited by 409

Massive accumulation of luminal protease-deficient axonal lysosomes at Alzheimer’s disease amyloid plaques

Gowrishankar, S., Yuan, P., Wu, Y., Schrag, M., Paradise, S., Grutzendler, J., De Camilli, P., & Ferguson, S. M. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1510329112 · Cited by 406

Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics

Whitwell, J. L., Weigand, S. D., Boeve, B. F., Senjem, M. L., Gunter, J. L., DeJesus-Hernandez, M., Rutherford, N. J., Baker, M., Knopman, D. S., Wszolek, Z. K., et al. (2012) · Brain · doi:10.1093/brain/aws001 · Cited by 397

Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members

Finch, N. A., Baker, M., Crook, R., Swanson, K. R., Kuntz, K., Surtees, R., Bisceglio, G. D., Rovelet‐Lecrux, A., Boeve, B. F., Petersen, R. C., et al. (2009) · Brain · doi:10.1093/brain/awn352 · Cited by 382

Progranulin Mediates Caspase-Dependent Cleavage of TAR DNA Binding Protein-43

Zhang, Y., Xu, Y.-F., Dickey, C. A., Buratti, E., Baralle, F. E., Bailey, R. M., Pickering-Brown, S. M., Dickson, D. W., & Petrucelli, L. (2007) · Journal of Neuroscience · doi:10.1523/jneurosci.3421-07.2007 · Cited by 371

Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies

Igaz, L. M., Kwong, L. K., Chen‐Plotkin, A. S., Winton, M. J., Unger, T. L., Xu, Y., Neumann, M., Trojanowski, J. Q., & Lee, V. M. ‐. (2009) · Journal of Biological Chemistry · doi:10.1074/jbc.m809462200 · Cited by 367

Proteinase 3 and neutrophil elastase enhance inflammation in mice by inactivating antiinflammatory progranulin

Kessenbrock, K., Fröhlich, L. F., Sixt, M., Lämmermann, T., Pfister, H., Bateman, A., Belaaouaj, A., Ring, J., Ollert, M. W., Fässler, R., et al. (2008) · Journal of Clinical Investigation · doi:10.1172/jci34694 · Cited by 363

Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study

Ber, I. L., Camuzat, A., Hannequin, D., Pasquier, F., Guedj, É., Rovelet‐Lecrux, A., Hahn‐Barma, V., van der Zee, J., Clot, F., Bakchine, S., et al. (2008) · Brain · doi:10.1093/brain/awn012 · Cited by 357

Cellular Localization of Gene Expression for Progranulin

DANIEL, R. L., He, Z., Carmichael, K. P., Halper, J. T., & Bateman, A. (2000) · Journal of Histochemistry & Cytochemistry · doi:10.1177/002215540004800713 · Cited by 346

Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury

Martens, L. H., Zhang, J., Barmada, S. J., Zhou, P., Kamiya, S., Sun, B., Min, S.-W., Gan, L., Finkbeiner, S. M., Huang, E. J., et al. (2012) · Journal of Clinical Investigation · doi:10.1172/jci63113 · Cited by 318

Whole-genome sequencing reveals important role for TBK1 and OPTN mutations in frontotemporal lobar degeneration without motor neuron disease

Pottier, C., Bieniek, K. F., Finch, N., van de Vorst, M., Baker, M., Perkersen, R., Brown, P., Ravenscroft, T., van Blitterswijk, M., Nicholson, A. M., et al. (2015) · Acta Neuropathologica · doi:10.1007/s00401-015-1436-x · Cited by 312

Low plasma progranulin levels predict progranulin mutations in frontotemporal lobar degeneration

Ghidoni, R., Benussi, L., Glionna, M., Franzoni, M., & Binetti, G. G. (2008) · Neurology · doi:10.1212/01.wnl.0000325058.10218.fc · Cited by 303

The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene

Mackenzie, I. R., Baker, M., Pickering-Brown, S. M., Hsiung, G. R., Lindholm, C., Dwosh, E., Gass, J. M., Cannon, A. D., Rademakers, R., Hutton, M., et al. (2006) · Brain · doi:10.1093/brain/awl271 · Cited by 300

Accelerated Lipofuscinosis and Ubiquitination in Granulin Knockout Mice Suggest a Role for Progranulin in Successful Aging

Ahmed, Z., Sheng, H., Xu, Y.-F., Lin, W.-L., Innes, A. E., Gass, J. M., Yu, X., Hou, H., Chiba, S., Yamanouchi, K., et al. (2010) · American Journal Of Pathology · doi:10.2353/ajpath.2010.090915 · Cited by 299

Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia

Rademakers, R., Eriksen, J. L., Baker, M. C., Robinson, T., Ahmed, Z., Lincoln, S., Finch, N. A., Rutherford, N. J., Crook, R., Josephs, K. A., et al. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn257 · Cited by 299

Differential roles of the ubiquitin proteasome system (UPS) and autophagy in the clearance of soluble and aggregated TDP-43 species

Scotter, E. L., Vance, C. A., Nishimura, A. L., Lee, Y., Chen, H.-J., Urwin, H., Sardone, V., Mitchell, J. C., Rogelj, B., Rubinsztein, D. C., et al. (2014) · Journal of Cell Science · doi:10.1242/jcs.140087 · Cited by 282

The genetics and neuropathology of frontotemporal lobar degeneration

Sieben, A., Van Langenhove, T., Engelborghs, S., Martin, J.-J., Boon, P., Cras, P., De Deyn, P.-P., Santens, P., Van Broeckhoven, C., & Cruts, M. (2012) · Acta Neuropathologica · doi:10.1007/s00401-012-1029-x · Cited by 280

TDP-43 functions and pathogenic mechanisms implicated in TDP-43 proteinopathies

Cohen, T. J., Lee, V. M. ‐., & Trojanowski, J. Q. (2011) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2011.06.004 · Cited by 271

C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis

Rohrer, J. D., Isaacs, A. M., Mizielinska, S., Mead, S., Lashley, T., Wray, S., Sidle, K. C. L., Fratta, P., Orrell, R. W., Hardy, J., et al. (2015) · The Lancet Neurology · doi:10.1016/s1474-4422(14)70233-9 · Cited by 255

Mutation within TARDBP leads to Frontotemporal Dementia without motor neuron disease

Borroni, B., Bonvicini, C., Alberici, A., Buratti, E., Agosti, C., Archetti, S., Papetti, A., Stuani, C., Luca, M. D., Gennarelli, M., et al. (2009) · Human Mutation · doi:10.1002/humu.21100 · Cited by 254

Isolation and sequence of the granulin precursor cDNA from human bone marrow reveals tandem cysteine-rich granulin domains.

Bhandari, V., Palfree, R. G. E., & Bateman, A. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.5.1715 · Cited by 245

TMEM106B regulates progranulin levels and the penetrance of FTLD in GRN mutation carriers

Finch, N., Carrasquillo, M. M., Baker, M., Rutherford, N. J., Coppola, G., DeJesus-Hernandez, M., Crook, R., Hunter, T., Ghidoni, R., Benussi, L., et al. (2011) · Neurology · doi:10.1212/wnl.0b013e31820a0e3b · Cited by 243

Distinct profiles of brain atrophy in frontotemporal lobar degeneration caused by progranulin and tau mutations

Rohrer, J. D., Ridgway, G. R., Marc Modat, A., Sebastien Ourselin, A., Mead, S., Fox, N. C., Rossor, M. N., & Warren, J. D. (2010) · NeuroImage · doi:10.1016/j.neuroimage.2009.12.088 · Cited by 241

Progranulin protects against amyloid β deposition and toxicity in Alzheimer's disease mouse models

Minami, S. S., Min, S.-W., Krabbe, G., Wang, C., Zhou, Y., Asgarov, R., Li, Y., Martens, L. H., Elia, L. P., Ward, M. E., et al. (2014) · Nature Medicine · doi:10.1038/nm.3672 · Cited by 237

Insights into the role of progranulin in immunity, infection, and inflammation

Jian, J., Konopka, J., & Liu, C.-J. (2012) · Journal of Leukocyte Biology · doi:10.1189/jlb.0812429 · Cited by 236

Novel Ubiquitin Neuropathology in Frontotemporal Dementia WithValosin-Containing ProteinGene Mutations

Forman, M. S., Mackenzie, I. R., Cairns, N. J., Swanson, E., Boyer, P. J., Drachman, D. A., Jhaveri, B. S., Karlawish, J. H., Pestronk, A., Smith, T. W., et al. (2006) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-200606000-00005 · Cited by 232

A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series

Beck, J. A., Rohrer, J. D., Campbell, T., Isaacs, A. M., Morrison, K. E., Goodall, G., Warrington, E. K., Stevens, J., Révész, T., Holton, J. L., et al. (2008) · Brain · doi:10.1093/brain/awm320 · Cited by 231

TMEM106B , the Risk Gene for Frontotemporal Dementia, Is Regulated by the microRNA-132/212 Cluster and Affects Progranulin Pathways

Chen-Plotkin, A. S., Unger, T. L., Gallagher, M. D., Bill, E., Kwong, L. K., Volpicelli-Daley, L., Busch, J. I., Akle, S., Grossman, M., Van Deerlin, V., et al. (2012) · The Journal of Neuroscience · doi:10.1523/jneurosci.0521-12.2012 · Cited by 230

Genetic contribution of FUS to frontotemporal lobar degeneration

Langenhove, T. V., van der Zee, J., Sleegers, K., Engelborghs, S., Vandenberghe, R. R., Gijselinck, I., den Broeck, M. V., Mattheijssens, M., Peeters, K., Deyn, P. P. D., et al. (2010) · Neurology · doi:10.1212/wnl.0b013e3181ccc732 · Cited by 225

Differential expression of 14 genes in amyotrophic lateral sclerosis spinal cord detected using gridded cDNA arrays

Malaspina, A., Kaushik, N. K., & de Belleroche, J. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.t01-1-00231.x · Cited by 212

Phenotypic variability associated with progranulin haploinsufficiency in patients with the common 1477C→T (Arg493X) mutation: an international initiative

Rademakers, R., Baker, M. C., Gass, J. M., Adamson, J. L., Huey, E. D., Momeni, P., Spina, S., Coppola, G., Karydas, A. M., Stewart, H. G., et al. (2007) · The Lancet Neurology · doi:10.1016/s1474-4422(07)70221-1 · Cited by 211

Toward Selective Histone Deacetylase Inhibitor Design: Homology Modeling, Docking Studies, and Molecular Dynamics Simulations of Human Class I Histone Deacetylases

Wang, D., Helquist, P. M., Wiech, N. L., & Wiest, O. (2005) · Journal of Medicinal Chemistry · doi:10.1021/jm0505011 · Cited by 211

Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: Implications for TDP-43 in the physiological response to neuronal injury

Moisse, K., Volkening, K., Leystra-Lantz, C., Welch, I., Hill, T., & Strong, M. J. (2009) · Brain Research · doi:10.1016/j.brainres.2008.10.021 · Cited by 210

Serum biomarker for progranulin‐associated frontotemporal lobar degeneration

Sleegers, K., Brouwers, N., Damme, P. V., Engelborghs, S., Gijselinck, I., van der Zee, J., Peeters, K., Mattheijssens, M., Cruts, M., Vandenberghe, R. R., et al. (2009) · Annals of Neurology · doi:10.1002/ana.21621 · Cited by 207

Purification of an autocrine growth factor homologous with mouse epithelin precursor from a highly tumorigenic cell line

Zhou, J., Gao, G., Crabb, J. W., & Serrero, G. (1993) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)82064-6 · Cited by 206

Granulins, a novel class of peptide from leukocytes

Bateman, A., Belcourt, D., Bennett, H. P. J., Lazure, C., & Solomon, S. (1990) · Biochemical and Biophysical Research Communications · doi:10.1016/s0006-291x(05)80908-8 · Cited by 206

The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function

Brady, O. A., Zheng, Y., Murphy, K. A., Huang, M. J., & Hu, F. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds475 · Cited by 205

Prosaposin facilitates sortilin-independent lysosomal trafficking of progranulin

Zhou, X., Sun, L., de Oliveira, F. M. B., Qi, X., Brown, W. J., Smolka, M. B., Sun, Y., & Hu, F. (2015) · The Journal of Cell Biology · doi:10.1083/jcb.201502029 · Cited by 204

Human tumors instigate granulin-expressing hematopoietic cells that promote malignancy by activating stromal fibroblasts in mice

Elkabets, M., Gifford, A. M., Scheel, C., Nilsson, B., Reinhardt, F., Bray, M.-A., Carpenter, A. E., Jirström, K., Magnusson, K., Ebert, B. L., et al. (2011) · Journal of Clinical Investigation · doi:10.1172/jci43757 · Cited by 201

SQSTM1Mutations in French Patients With Frontotemporal Dementia or Frontotemporal Dementia With Amyotrophic Lateral Sclerosis

Ber, I. L. (2013) · JAMA Neurology · doi:10.1001/jamaneurol.2013.3849 · Cited by 198

The Spectrum of Mutations in Progranulin: A Collaborative Study Screening 545 Cases of Neurodegeneration

Yu, C.-E., Bird, T. D., Bekris, L. M., Montine, T. J., Leverenz, J. B., Steinbart, E., Galloway, N. M., Feldman, H., Woltjer, R., Miller, C. A., et al. (2010) · Archives of Neurology · doi:10.1001/archneurol.2009.328 · Cited by 190

Association of TMEM106B Gene Polymorphism With Age at Onset in Granulin Mutation Carriers and Plasma Granulin Protein Levels

Cruchaga, C., Graff, C., Chiang, H., Wang, J., Hinrichs, A. L., Spiegel, N., Bertelsen, S. E., Mayo, K., Norton, J. B., Morris, J. C., et al. (2011) · Archives of Neurology · doi:10.1001/archneurol.2010.350 · Cited by 189

Alteration of behavioural phenotype in mice by targeted disruption of the progranulin gene

Kayasuga, Y., Chiba, S., Suzuki, M., Kikusui, T., Matsuwaki, T., Yamanouchi, K., Kotaki, H., Horai, R., Iwakura, Y., & Nishihara, M. (2007) · Behavioural Brain Research · doi:10.1016/j.bbr.2007.07.020 · Cited by 189

The epithelin precursor encodes two proteins with opposing activities on epithelial cell growth.

Plowman, G. D., Green, J. M., Neubauer, M., Buckley, S., McDonald, V. L., Todaro, G. J., & SHOYAB, M. (1992) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)42382-4 · Cited by 187

HDDD2 is a familial frontotemporal lobar degeneration with ubiquitin‐positive, tau‐negative inclusions caused by a missense mutation in the signal peptide of progranulin

Mukherjee, O., Pástor, P., Cairns, N. J., Chakraverty, S., Kauwe, J. S. K., Shears, S., Behrens, M. I., Budde, J., Hinrichs, A. L., Norton, J. B., et al. (2006) · Annals of Neurology · doi:10.1002/ana.20963 · Cited by 187

Biological activities and signaling pathways of the granulin/epithelin precursor.

Zanocco‐Marani, T., Bateman, A., Romano, G., Valentinis, B., He, Z., & Baserga, R. (1999) · PubMed · Cited by 184

Neuropathologic Features of Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions With Progranulin Gene (PGRN) Mutations

Josephs, K. A., Ahmed, Z., Katsuse, O., Parisi, J. F., Boeve, B. F., Knopman, D. S., Petersen, R. C., Davies, P., Duara, R., Graff‐Radford, N. R., et al. (2007) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e31803020cf · Cited by 184

miR-107 Regulates Granulin/Progranulin with Implications for Traumatic Brain Injury and Neurodegenerative Disease

Wang, W., Wilfred, B. R., Madathil, S. K., Tang, G., Hu, Y., Dimayuga, J., Stromberg, A. J., Huang, Q., Saatman, K. E., & Nelson, P. T. (2010) · American Journal Of Pathology · doi:10.2353/ajpath.2010.091202 · Cited by 183

Granulin Is a Soluble Cofactor for Toll-like Receptor 9 Signaling

Park, B., Buti, L., Lee, S., Matsuwaki, T., Spooner, E., Brinkmann, M. M., Nishihara, M., & Ploegh, H. L. (2011) · Immunity · doi:10.1016/j.immuni.2011.01.018 · Cited by 182

Behavioral deficits and progressive neuropathology in progranulin-deficient mice: a mouse model of frontotemporal dementia

Yin, F., Dumont, M., Banerjee, R., Ma, Y., Li, H., Lin, M. T., Beal, M. F., Nathan, C., Thomas, B., & Ding, A. (2010) · The FASEB Journal · doi:10.1096/fj.10-161471 · Cited by 180

Epithelins 1 and 2: isolation and characterization of two cysteine-rich growth-modulating proteins.

SHOYAB, M., McDonald, V. L., Byles, C. A., Todaro, G. J., & Plowman, G. D. (1990) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.87.20.7912 · Cited by 179

Dissociation of Frontotemporal Dementia–Related Deficits and Neuroinflammation in Progranulin Haploinsufficient Mice

Filiano, A. J., Martens, L. H., Young, A. H., Warmus, B. A., Zhou, P., Diaz‐Ramirez, G., Jiao, J., Zhang, Z., Huang, E. J., Gao, F., et al. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.6103-11.2013 · Cited by 175

Synaptic dysfunction in progranulin-deficient mice

Petkau, T. L., Neal, S. J., Milnerwood, A. J., Mew, A., Hill, A. M., Orban, P. C., Gregg, J., Lu, G., Feldman, H., Mackenzie, I. R., et al. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2011.10.016 · Cited by 175

The hairpin stack fold, a novel protein architecture for a new family of protein growth factors

Hrabal, R., Chen, Z., James, S., Bennett, H. P. J., & Ni, F. (1996) · Nature Structural & Molecular Biology · doi:10.1038/nsb0996-747 · Cited by 174

Progranulin gene expression regulates epithelial cell growth and promotes tumor growth in vivo.

He, Z., & Bateman, A. (1999) · PubMed · Cited by 171

Progranulin null mutations in both sporadic and familial frontotemporal dementia

Ber, I. L., van der Zee, J., Hannequin, D., Gijselinck, I., Campion, D., Puel, M., Laquerrière, A., Pooter, T. D., Camuzat, A., den Broeck, M. V., et al. (2007) · Human Mutation · doi:10.1002/humu.20520 · Cited by 170

TDP-43 frontotemporal lobar degeneration and autoimmune disease

Miller, Z. A., Rankin, K. P., Graff-Radford, N. R., Takada, L. T., Sturm, V. E., Cleveland, C. M., Criswell, L. A., Jaeger, P. A., Stan, T., Heggeli, K. A., et al. (2013) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2012-304644 · Cited by 170

Missense Mutations in the Progranulin Gene Linked to Frontotemporal Lobar Degeneration with Ubiquitin-immunoreactive Inclusions Reduce Progranulin Production and Secretion

Shankaran, S. S., Capell, A., Hruscha, A., Fellerer, K., Neumann, M., Schmid, B., & Haass, C. (2007) · Journal of Biological Chemistry · doi:10.1074/jbc.m705115200 · Cited by 168

Proteolytic processing of TAR DNA binding protein‐43 by caspases produces C‐terminal fragments with disease defining properties independent of progranulin

Dormann, D., Capell, A., Carlson, A. M., Shankaran, S. S., Rodde, R., Neumann, M., Kremmer, E., Matsuwaki, T., Yamanouchi, K., Nishihara, M., et al. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06211.x · Cited by 166

Membrane Orientation and Subcellular Localization of Transmembrane Protein 106B (TMEM106B), a Major Risk Factor for Frontotemporal Lobar Degeneration

Lang, C. M., Fellerer, K., Schwenk, B. M., Kuhn, P., Kremmer, E., Edbauer, D., Capell, A., & Haass, C. (2012) · Journal of Biological Chemistry · doi:10.1074/jbc.m112.365098 · Cited by 165

TDP-43 in aging and Alzheimer's disease - a review.

Wilson, A. C., Dugger, B. N., Dickson, D. W., & Wang, D.-S. (2011) · PubMed Central · Cited by 164

Genetic variability in progranulin contributes to risk for clinically diagnosed Alzheimer disease

Brouwers, N., Sleegers, K., Engelborghs, S., Maurer‐Stroh, S., Gijselinck, I., van der Zee, J., Pickut, B. A., den Broeck, M. V., Mattheijssens, M., Peeters, K., et al. (2008) · Neurology · doi:10.1212/01.wnl.0000319688.89790.7a · Cited by 162

Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease

Cairns, N. J., Grossman, M., Arnold, S. E., Burn, D. J., Jaros, E., Perry, R. H., Duyckaerts, C. J., Stankoff, B., Pillon, B., Skullerud, K., et al. (2004) · Neurology · doi:10.1212/01.wnl.0000139809.16817.dd · Cited by 160

Pharmacoproteomics of a Metalloproteinase Hydroxamate Inhibitor in Breast Cancer Cells: Dynamics of Membrane Type 1 Matrix Metalloproteinase-Mediated Membrane Protein Shedding

Butler, G. S., Dean, R. A., Tam, E. M., & Overall, C. M. (2008) · Molecular and Cellular Biology · doi:10.1128/mcb.01775-07 · Cited by 159

Progranulin is expressed within motor neurons and promotes neuronal cell survival

Ryan, C. L., Baranowski, D. C., Chitramuthu, B. P., Malik, S., Li, Z., Cao, M., Minotti, S., Durham, H. D., Kay, D. G., Shaw, C. A., et al. (2009) · BMC Neuroscience · doi:10.1186/1471-2202-10-130 · Cited by 158

Progranulin regulates neuronal outgrowth independent of Sortilin

Gass, J. M., Lee, C. W., Cook, C. N., Finch, N. A., Stetler, C. T., Jansen‐West, K. R., Lewis, J. M., Link, C. D., Rademakers, R., Nykjær, A., et al. (2012) · Molecular Neurodegeneration · doi:10.1186/1750-1326-7-33 · Cited by 158

Structure dissection of human progranulin identifies well‐folded granulin/epithelin modules with unique functional activities

Tolkatchev, D., Malik, S., Vinogradova, A., Wang, P., Chen, Z., Xu, P., Bennett, H. P. J., Bateman, A., & Ni, F. (2008) · Protein Science · doi:10.1110/ps.073295308 · Cited by 157

Therapy and clinical trials in frontotemporal dementia: past, present, and future

Tsai, R. M., & Boxer, A. L. (2016) · Journal of Neurochemistry · doi:10.1111/jnc.13640 · Cited by 157

Progranulin is Neurotrophic In Vivo and Protects against a Mutant TDP-43 Induced Axonopathy

Laird, A. S., Hoecke, A. V., Muynck, L. D., Timmers, M., Bosch, L. V. D., Damme, P. V., & Robberecht, W. (2010) · PLoS ONE · doi:10.1371/journal.pone.0013368 · Cited by 154

Progranulin (PC-cell-derived growth factor/acrogranin) regulates invasion and cell survival.

He, Z., Ismail, A., Kriazhev, L., Sadvakassova, G., & Bateman, A. (2002) · PubMed · Cited by 153

Progranulin (acrogranin/PC cell‐derived growth factor/granulin‐epithelin precursor) is expressed in the placenta, epidermis, microvasculature, and brain during murine development

DANIEL, R. L., Daniels, E., He, Z., & Bateman, A. (2003) · Developmental Dynamics · doi:10.1002/dvdy.10341 · Cited by 153

Increased lysosomal biogenesis in activated microglia and exacerbated neuronal damage after traumatic brain injury in progranulin-deficient mice

Tanaka, Y., Matsuwaki, T., Yamanouchi, K., & Nishihara, M. (2013) · Neuroscience · doi:10.1016/j.neuroscience.2013.06.049 · Cited by 153

Progranulin expression in the developing and adult murine brain

Petkau, T. L., Neal, S. J., Orban, P. C., MacDonald, J. L., Hill, A. M., Lu, G., Feldman, H. H., Mackenzie, I. R. A., & Leavitt, B. R. (2010) · Journal of Comparative Neurology · doi:10.1002/cne.22430 · Cited by 152

Suberoylanilide Hydroxamic Acid (Vorinostat) Up-regulates Progranulin Transcription

Cenik, B., Sephton, C. F., Dewey, C. M., Xian, X., Wei, S., Yu, K., Niu, W., Coppola, G., Coughlin, S. E., Lee, S. E., et al. (2011) · Journal of Biological Chemistry · doi:10.1074/jbc.m110.193433 · Cited by 150

Alzheimer and Parkinson Diagnoses in Progranulin Null Mutation Carriers in an Extended Founder Family

Brouwers, N., Nuytemans, K., van der Zee, J., Gijselinck, I., Engelborghs, S., Theuns, J., Kumar‐Singh, S., Pickut, B. A., Pals, P., Dermaut, B., et al. (2007) · Archives of Neurology · doi:10.1001/archneur.64.10.1436 · Cited by 150

Mediation of estrogen mitogenic effect in human breast cancer MCF-7 cells by PC-cell-derived growth factor (PCDGF/granulin precursor)

Lu, R., & Serrero, G. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.98.1.142 · Cited by 146

Granulin-Epithelin Precursor and ATP-Dependent Binding Cassette (ABC)B5 Regulate Liver Cancer Cell Chemoresistance

Cheung, S., Cheung, P. F., Cheng, C. K. C., Wong, N. C. L., & Fan, S. T. (2010) · Gastroenterology · doi:10.1053/j.gastro.2010.07.049 · Cited by 142

A Reassessment of the Neuropathology of Frontotemporal Dementia Linked to Chromosome 3

Holm, I. E., Englund, E. M., Mackenzie, I. R., Johannsen, P., & Isaacs, A. M. (2007) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e3181567f02 · Cited by 141

Proepithelin Promotes Migration and Invasion of 5637 Bladder Cancer Cells through the Activation of ERK1/2 and the Formation of a Paxillin/FAK/ERK Complex

Monamì, G., Gonzalez, E. M., Hellman, M., Gomella, L. G., Baffa, R., Iozzo, R. V., & Morrione, A. (2006) · Cancer Research · doi:10.1158/0008-5472.can-06-0633 · Cited by 141

Induced pluripotent stem cell models of progranulin-deficient frontotemporal dementia uncover specific reversible neuronal defects

Almeida, S., Zhang, Z., Coppola, G., Mao, W., Futai, K., Karydas, A., Geschwind, M. D., Tartaglia, M. C., Gao, F., Gianni, D., et al. (2012) · Cell Reports · doi:10.1016/j.celrep.2012.09.007 · Cited by 140

Regulation of Progranulin Expression in Human Microglia and Proteolysis of Progranulin by Matrix Metalloproteinase-12 (MMP-12)

Suh, H.-S., Choi, N., Tarassishin, L., & Lee, S. C. (2012) · PLoS ONE · doi:10.1371/journal.pone.0035115 · Cited by 139

Lysosome size, motility and stress response regulated by fronto-temporal dementia modifier TMEM106B

Stagi, M., Klein, Z. A., Gould, T. J., Bewersdorf, J., & Strittmatter, S. M. (2014) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2014.07.006 · Cited by 137

Cartilage Oligomeric Matrix Protein Associates with Granulin-Epithelin Precursor (GEP) and Potentiates GEP-stimulated Chondrocyte Proliferation

Xu, K., Zhang, Y., Ilalov, K., Carlson, C. S., Feng, J. Q., Cesare, P. E. D., & Liu, C.-J. (2007) · Journal of Biological Chemistry · doi:10.1074/jbc.m608744200 · Cited by 134

TDP-43-mediated neurodegeneration: towards a loss-of-function hypothesis?

Broeck, L. V., Callaerts, P., & Dermaut, B. (2013) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2013.11.003 · Cited by 134

Progranulin mutations and amyotrophic lateral sclerosis or amyotrophic lateral sclerosis-frontotemporal dementia phenotypes

Schymick, J. C., Yang, Y., Andersen, P. M., Vonsattel, J. P., Greenway, M. J., Momeni, P., Elder, J., Chiò, A., Restagno, G., Robberecht, W., et al. (2006) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2006.109553 · Cited by 134

The growth factor progranulin attenuates neuronal injury induced by cerebral ischemia-reperfusion through the suppression of neutrophil recruitment

Egashira, Y., Suzuki, Y., Azuma, Y., Takagi, T., Mishiro, K., Sugitani, S., Tsuruma, K., Shimazawa, M., Yoshimura, S., Kashimata, M., et al. (2013) · Journal of Neuroinflammation · doi:10.1186/1742-2094-10-105 · Cited by 134

Progranulin Mutations as Risk Factors for Alzheimer Disease

Perry, D. C., Lehmann, M., Yokoyama, J. S., Karydas, A., Lee, J. J., Coppola, G., Grinberg, L. T., Geschwind, D. H., Seeley, W. W., Miller, B. L., et al. (2013) · JAMA Neurology · doi:10.1001/2013.jamaneurol.393 · Cited by 133

Frontotemporal Lobar Degeneration: Epidemiology, Pathology, Diagnosis and Management

Seltman, R. E., & Matthews, B. R. (2012) · CNS Drugs · doi:10.2165/11640070-000000000-00000 · Cited by 132

Neurotrophins and their receptors in early development of the mammalian nervous system

Bartkowska, K., Turlejski, K., & Djavadian, R. L. (2010) · Acta Neurobiologiae Experimentalis · doi:10.55782/ane-2010-1816 · Cited by 132

Rescue of Progranulin Deficiency Associated with Frontotemporal Lobar Degeneration by Alkalizing Reagents and Inhibition of Vacuolar ATPase

Capell, A., Liebscher, S., Fellerer, K., Brouwers, N., Willem, M., Lammich, S., Gijselinck, I., Bittner, T., Carlson, A. M., Sasse, F., et al. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.5757-10.2011 · Cited by 131

Progranulin promotes neurite outgrowth and neuronal differentiation by regulating GSK-3β

Gao, X., Joselin, A. P., Wang, L., Kar, A. N., Ray, P., Bateman, A., Goate, A., & Wu, J. Y. (2010) · Protein & Cell · doi:10.1007/s13238-010-0067-1 · Cited by 131

Progranulin: A growth factor, a novel TNFR ligand and a drug target

Liu, C.-J., & Bosch, X. (2011) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2011.10.003 · Cited by 131

Structure, Function, and Mechanism of Progranulin; the Brain and Beyond

Toh, H., Chitramuthu, B. P., Bennett, H. P. J., & Bateman, A. (2011) · Journal of Molecular Neuroscience · doi:10.1007/s12031-011-9569-4 · Cited by 130

Genome-wide Screen Identifies rs646776 near Sortilin as a Regulator of Progranulin Levels in Human Plasma

Carrasquillo, M. M., Nicholson, A. M., Finch, N., Gibbs, J. R., Baker, M., Rutherford, N. J., Hunter, T. A., DeJesus-Hernandez, M., Bisceglio, G. D., Mackenzie, I. R., et al. (2010) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2010.11.002 · Cited by 130

EphA2 is a functional receptor for the growth factor progranulin

Neill, T., Buraschi, S., Goyal, A. K., Sharpe, C., Natkanski, E., Schaefer, L., Morrione, A., & Iozzo, R. V. (2016) · The Journal of Cell Biology · doi:10.1083/jcb.201603079 · Cited by 130

Differential Aging Analysis in Human Cerebral Cortex Identifies Variants in TMEM106B and GRN that Regulate Aging Phenotypes

Rhinn, H., & Abeliovich, A. (2017) · Cell Systems · doi:10.1016/j.cels.2017.02.009 · Cited by 130

Phenotypic Heterogeneity of Monogenic Frontotemporal Dementia

Benussi, A., Padovani, A., & Borroni, B. (2015) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2015.00171 · Cited by 129

Cellular ageing, increased mortality and FTLD‐TDP‐associated neuropathology in progranulin knockout mice

Wils, H., Kleinberger, G., Pereson, S., Janssens, J., Capell, A., Dam, D. V., Cuijt, I., Joris, G., Deyn, P. P. D., Haass, C., et al. (2012) · The Journal of Pathology · doi:10.1002/path.4043 · Cited by 128

Functional Genomic Analyses Identify Pathways Dysregulated by Progranulin Deficiency, Implicating Wnt Signaling

Rosen, E. Y., Wexler, E. M., Versano, R., Coppola, G., Gao, F., Winden, K. D., Oldham, M. C., Martens, L. H., Zhou, P., Farese, R. V., et al. (2011) · Neuron · doi:10.1016/j.neuron.2011.07.021 · Cited by 128

Core features of frontotemporal dementia recapitulated in progranulin knockout mice

Ghoshal, N., Dearborn, J. T., Wozniak, D. F., & Cairns, N. J. (2012) · Neurobiology of Disease · doi:10.1016/j.nbd.2011.08.029 · Cited by 127

TMEM106B is associated with frontotemporal lobar degeneration in a clinically diagnosed patient cohort

van der Zee, J., Langenhove, T. V., Kleinberger, G., Sleegers, K., Engelborghs, S., Vandenberghe, R. R., Santens, P., den Broeck, M. V., Joris, G., Brys, J., et al. (2011) · Brain · doi:10.1093/brain/awr007 · Cited by 127

Voxel-based morphometry patterns of atrophy in FTLD with mutations in MAPT or PGRN

Whitwell, J. L., Jack, C. R., Boeve, B. F., Senjem, M. L., Baker, M., Rademakers, R., Ivnik, R. J., Knopman, D. S., Wszołek, Z. K., Petersen, R. C., et al. (2009) · Neurology · doi:10.1212/01.wnl.0000343851.46573.67 · Cited by 126

Brain atrophy over time in genetic and sporadic frontotemporal dementia: a study of 198 serial magnetic resonance images

Whitwell, J. L., Boeve, B. F., Weigand, S. D., Senjem, M. L., Gunter, J. L., Baker, M., DeJesus‐Hernandez, M., Knopman, D. S., Wszolek, Z. K., Petersen, R. C., et al. (2015) · European Journal of Neurology · doi:10.1111/ene.12675 · Cited by 125

PC cell-derived growth factor (PCDGF/GP88, progranulin) stimulates migration, invasiveness and VEGF expression in breast cancer cells

Tangkeangsirisin, W. (2004) · Carcinogenesis · doi:10.1093/carcin/bgh171 · Cited by 125

Targeted manipulation of the sortilin–progranulin axis rescues progranulin haploinsufficiency

Lee, W. C., Almeida, S., Prudencio, M., Caulfield, T. R., Zhang, Y.-J., Tay, W. M., Bauer, P. O., Chew, J., Sasaguri, H., Jansen-West, K. R., et al. (2014) · Human Molecular Genetics · doi:10.1093/hmg/ddt534 · Cited by 125

Portuguese family with the co-occurrence of frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis phenotypes due to progranulin gene mutation

Almeida, M. R., Macário, M. C., Ramos, L., Baldeiras, I., Ribeiro, M. H., & Santana, I. (2016) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2016.02.019 · Cited by 125

A Novel Interaction between Perlecan Protein Core and Progranulin

González, E. M., Mongiat, M., Slater, S. J., Baffa, R., & Iozzo, R. V. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.c300310200 · Cited by 124

Frontotemporal lobar degeneration: current perspectives

Kurz, A., Riedl, L., Mackenzie, I. R., Förstl, H., & Diehl‐Schmid, J. (2014) · Neuropsychiatric Disease and Treatment · doi:10.2147/ndt.s38706 · Cited by 123

Identification of a human glioma-associated growth factor gene, granulin, using differential immuno-absorption.

Liau, L. M., Lallone, R. L., Seitz, R. S., Buznikov, A. G., Gregg, J. P., Kornblum, H. I., Nelson, S. F., & Bronstein, J. M. (2000) · PubMed · Cited by 123

Exacerbated inflammatory responses related to activated microglia after traumatic brain injury in progranulin-deficient mice

Tanaka, Y., Matsuwaki, T., Yamanouchi, K., & Nishihara, M. (2012) · Neuroscience · doi:10.1016/j.neuroscience.2012.11.032 · Cited by 120

Granulin–Epithelin Precursor Overexpression Promotes Growth and Invasion of Hepatocellular Carcinoma

Cheung, S., Wong, S. Y., Leung, K. L., Chen, X., So, S. K., Ng, I. O., & Fan, S. T. (2004) · Clinical Cancer Research · doi:10.1158/1078-0432.ccr-04-0960 · Cited by 120

Genetic and Clinical Features of Progranulin-Associated Frontotemporal Lobar Degeneration

Chen-Plotkin, A. S., Martinez-Lage, M., Sleiman, P. M. A., Hu, W., Greene, R., Wood, E. M., Bing, S., Grossman, M., Schellenberg, G. D., Hatanpaa, K. J., et al. (2011) · Archives of Neurology · doi:10.1001/archneurol.2011.53 · Cited by 120

Cellular Effects of Progranulin in Health and Disease

Muynck, L. D., & Damme, P. V. (2011) · Journal of Molecular Neuroscience · doi:10.1007/s12031-011-9553-z · Cited by 118

A neurodegenerative disease mutation that accelerates the clearance of apoptotic cells

Kao, A. W., Eisenhut, R. J., Martens, L. H., Nakamura, A., Huang, A., Bagley, J. A., Zhou, P., de Luis, A., Neukomm, L. J., Cabello, J., et al. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1100650108 · Cited by 116

Multiple therapeutic effects of progranulin on experimental acute ischaemic stroke

Kanazawa, M., Kawamura, K., Takahashi, T., Miura, M., Tanaka, Y., Koyama, M., Toriyabe, M., Igarashi, H., Nakada, T., Nishihara, M., et al. (2015) · Brain · doi:10.1093/brain/awv079 · Cited by 116

Granulin mutation drives brain damage and reorganization from preclinical to symptomatic FTLD

Borroni, B., Alberici, A., Cercignani, M., Premi, E., Serra, L., Cerini, C., Cosseddu, M., Pettenati, C., Turla, M., Archetti, S., et al. (2011) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2011.10.031 · Cited by 113

Prevention of LPS-Induced Acute Lung Injury in Mice by Progranulin

Guo, Z., Li, Q., Yang, H., Liang, Y., Xu, Z., & Ren, T. (2012) · Mediators of Inflammation · doi:10.1155/2012/540794 · Cited by 113

Transcriptional gene network inference from a massive dataset elucidates transcriptome organization and gene function

Belcastro, V., Siciliano, V., Gregoretti, F., Mithbaokar, P., Dharmalingam, G., Berlingieri, S., Iorio, F., Oliva, G., Polishchuck, R. S., Brunetti‐Pierri, N., et al. (2011) · Nucleic Acids Research · doi:10.1093/nar/gkr593 · Cited by 112

ADAMTS-7, a Direct Target of PTHrP, Adversely Regulates Endochondral Bone Growth by Associating with and Inactivating GEP Growth Factor

Bai, X.-H., Wang, D., Kong, L., Zhang, Y., Luan, Y., Kobayashi, T., Kronenberg, H. M., Yu, X., & Liu, C.-J. (2009) · Molecular and Cellular Biology · doi:10.1128/mcb.00056-09 · Cited by 111

Progranulin Does Not Bind Tumor Necrosis Factor (TNF) Receptors and Is Not a Direct Regulator of TNF-Dependent Signaling or Bioactivity in Immune or Neuronal Cells

Chen, X., Chang, J., Deng, Q., Xu, J., Nguyen, T. A., Martens, L. H., Cenik, B., Taylor, G., Hudson, K. F., Chung, J., et al. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.5336-12.2013 · Cited by 110

Progranulin: A promising therapeutic target for rheumatoid arthritis

Liu, C.-J. (2011) · FEBS Letters · doi:10.1016/j.febslet.2011.04.065 · Cited by 109

Extracellular progranulin protects cortical neurons from toxic insults by activating survival signaling

Xu, J., Xilouri, M., Bruban, J., Shioi, J., Shao, Z., Papazoglou, I., Vekrellis, K., & Robakis, N. K. R. K. (2011) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2011.06.017 · Cited by 109

Trehalose upregulates progranulin expression in human and mouse models of GRN haploinsufficiency: a novel therapeutic lead to treat frontotemporal dementia

Holler, C. J., Taylor, G., McEachin, Z. T., Deng, Q., Watkins, W. J., Hudson, K. F., Easley, C. A., Hu, W. T., Hales, C. M., Rossoll, W., et al. (2016) · Molecular Neurodegeneration · doi:10.1186/s13024-016-0114-3 · Cited by 109

Progranulin protects against renal ischemia/reperfusion injury in mice

Zhou, M., Tang, W., Fu, Y., Xu, X., Wang, Z., Lu, Y., Liu, F., Yang, X., Wei, X., Zhang, Y., et al. (2015) · Kidney International · doi:10.1038/ki.2014.403 · Cited by 107

Progranulin is a chemoattractant for microglia and stimulates their endocytic activity

Pickford, F., Marcus, J., Camargo, L. M., Xiao, Q., Graham, D., Mo, J.-R., Burkhardt, M., Kulkarni, V., Crispino, J., Hering, H., et al. (2011) · The American Journal of Pathology · doi:10.1016/j.ajpath.2010.11.002 · Cited by 107 · cited 1×

C-Terminus of Progranulin Interacts with the Beta-Propeller Region of Sortilin to Regulate Progranulin Trafficking

Zheng, Y., Brady, O. A., Meng, P. S., Mao, Y., & Hu, F. (2011) · PLoS ONE · doi:10.1371/journal.pone.0021023 · Cited by 105

Granulin-epithelin precursor as a therapeutic target for hepatocellular carcinoma

Ho, J. C.-Y., Ip, Y. C., Cheung, S., Lee, Y. T., Chan, K. F., Wong, S. Y., & Fan, S. T. (2008) · Hepatology · doi:10.1002/hep.22191 · Cited by 105

Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD

Ward, M. E., Taubes, A. L., Chen, R. Y., Miller, B. L., Sephton, C. F., Gelfand, J. M., Minami, S., Boscardin, W. J., Martens, L. H., Seeley, W. W., et al. (2014) · The Journal of Experimental Medicine · doi:10.1084/jem.20140214 · Cited by 105

Quantification of the Relative Contributions of Loss-of-function and Gain-of-function Mechanisms in TAR DNA-binding Protein 43 (TDP-43) Proteinopathies

Cascella, R., Capitini, C., Fani, G., Dobson, C. M., Cecchi, C., & Chiti, F. (2016) · Journal of Biological Chemistry · doi:10.1074/jbc.m116.737726 · Cited by 101

Common Variant in GRN Is a Genetic Risk Factor for Hippocampal Sclerosis in the Elderly

Dickson, D. W., Baker, M., & Rademakers, R. (2010) · Neurodegenerative Diseases · doi:10.1159/000289231 · Cited by 100

Brain Magnetic Resonance Imaging Structural Changes in a Pedigree of Asymptomatic Progranulin Mutation Carriers

Borroni, B., Alberici, A., Premi, E., Archetti, S., Garibotto, V., Agosti, C., Gasparotti, R., Luca, M. D., Perani, D., & Padovani, A. (2008) · Rejuvenation Research · doi:10.1089/rej.2007.0623 · Cited by 99

Progranulin Deficiency Promotes Post-Ischemic Blood–Brain Barrier Disruption

Jackman, K. A., Kahles, T., Lane, D., García‐Bonilla, L., Abe, T., Capone, C., Hochrainer, K., Voss, H. U., Zhou, P., Ding, A. H., et al. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.4318-13.2013 · Cited by 99

Trehalose decreases mutant SOD1 expression and alleviates motor deficiency in early but not end-stage amyotrophic lateral sclerosis in a SOD1-G93A mouse model

Li, Y., Guo, Y., wang, X., Yu, X., Duan, W., Hong, K., Wang, J., Han, H., & Li, C. (2015) · Neuroscience · doi:10.1016/j.neuroscience.2015.03.061 · Cited by 98

Loss of function mutations in the progranulin gene are related to pro-inflammatory cytokine dysregulation in frontotemporal lobar degeneration patients

Bossù, P., Salani, F., Alberici, A., Archetti, S., Bellelli, G., Galimberti, D., Scarpini, E. A., Spalletta, G., Caltagirone, C., Padovani, A., et al. (2011) · Journal of Neuroinflammation · doi:10.1186/1742-2094-8-65 · Cited by 97

Analyses of the MAPT, PGRN, and C9orf72 mutations in Japanese patients with FTLD, PSP, and CBS

Ogaki, K., Li, Y., Takanashi, M., Ishikawa, K., Kobayashi, T., Nonaka, T., Hasegawa, M., Kishi, M., Yoshino, H., Funayama, M., et al. (2012) · Parkinsonism & Related Disorders · doi:10.1016/j.parkreldis.2012.06.019 · Cited by 96

Trehalose intake induces chaperone molecules along with autophagy in a mouse model of Lewy body disease

Tanji, K., Miki, Y., Maruyama, A., Mimura, J., Matsumiya, T., Mori, F., Imaizumi, T., Itoh, K., & Wakabayashi, K. (2015) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2015.08.076 · Cited by 94

Progranulin locus deletion in frontotemporal dementia

Gijselinck, I., van der Zee, J., Engelborghs, S., Goossens, D., Peeters, K., Mattheijssens, M., Corsmit, E., Del‐Favero, J., Deyn, P. P. D., Broeckhoven, C. V., et al. (2007) · Human Mutation · doi:10.1002/humu.20651 · Cited by 94

Prosaposin is a regulator of progranulin levels and oligomerization

Nicholson, A. M., Finch, N. A., Almeida, M., Perkerson, R. B., van Blitterswijk, M., Wojtas, A., Cenik, B., Rotondo, S., Inskeep, V., Almasy, L., et al. (2016) · Nature Communications · doi:10.1038/ncomms11992 · Cited by 91

Restoring neuronal progranulin reverses deficits in a mouse model of frontotemporal dementia

Arrant, A. E., Filiano, A. J., Unger, D. E., Young, A. H., & Roberson, E. D. (2017) · Brain · doi:10.1093/brain/awx060 · Cited by 90

MicroRNA-29b Regulates the Expression Level of Human Progranulin, a Secreted Glycoprotein Implicated in Frontotemporal Dementia

Jiao, J., Herl, L. D., Farese, R. V., & Gao, F. (2010) · PLoS ONE · doi:10.1371/journal.pone.0010551 · Cited by 89

Progranulin Deficiency Decreases Gross Neural Connectivity But Enhances Transmission at Individual Synapses

Tapia, L., Milnerwood, A. J., Guo, A., Mills, F., Yoshida, E., Vasuta, C., Mackenzie, I. R., Raymond, L. A., Cynader, M. S., Jia, W. W.-G., et al. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.6244-10.2011 · Cited by 87

Corticobasal Syndrome Associated With the A9D Progranulin Mutation

Spina, S., Murrell, J. R., Huey, E. D., Wassermann, E. M., Pietrini, P., Grafman, J. H., & Ghetti, B. F. (2007) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e3181567873 · Cited by 87

rs5848 polymorphism and serum progranulin level

Hsiung, G. R., Fok, A., Feldman, H., Rademakers, R., & Mackenzie, I. R. (2010) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2010.10.009 · Cited by 87

Progranulin expression correlates with dense‐core amyloid plaque burden in Alzheimer disease mouse models

Pereson, S., Wils, H., Kleinberger, G., McGowan, E. M., Vandewoestyne, M., Broeck, B. V., Joris, G., Cuijt, I., Deforce, D. L. D., Hutton, M. L., et al. (2009) · The Journal of Pathology · doi:10.1002/path.2580 · Cited by 84

Progranulin plasma levels as potential biomarker for the identification of GRN deletion carriers. A case with atypical onset as clinical amnestic Mild Cognitive Impairment converted to Alzheimer's disease

Carecchio, M., Fenoglio, C., Riz, M. A. D., Guidi, I., Comi, C., Cortini, F., Venturelli, E., Restelli, I., Cantoni, C., Bresolin, N., et al. (2009) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2009.07.011 · Cited by 84

Amyloid ß and Tau Alzheimer¿s disease related pathology is reduced by toll-like receptor 9 stimulation

Scholtzova, H., Chianchiano, P. E., Pan, J., Sun, Y., Goñi, F., Mehta, P. D., & Wısnıewskı, T. (2014) · Acta Neuropathologica Communications · doi:10.1186/s40478-014-0101-2 · Cited by 84

Pathogenic cysteine mutations affect progranulin function and production of mature granulins

Wang, J., Damme, P. V., Cruchaga, C., Gitcho, M. A., Vidal-Taboada, J. M., Seijo‐Martínez, M., Wang, L., Wu, J. Y., Robberecht, W., & Goate, A. (2009) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2009.06546.x · Cited by 83

Anterior brain glucose hypometabolism predates dementia in progranulin mutation carriers

Jacova, C., Hsiung, G. R., Tawankanjanachot, I., Dinelle, K., McCormick, S., Gonzalez, M., Lee, H., Sengdy, P., Bouchard‐Kerr, P., Baker, M., et al. (2013) · Neurology · doi:10.1212/wnl.0b013e3182a8237e · Cited by 83

Microglial Upregulation of Progranulin as a Marker of Motor Neuron Degeneration

Philips, T., Muynck, L. D., Thu, H. N. T., Weynants, B., Vanacker, P., Dhondt, J., Sleegers, K., Schelhaas, H. J., Verbeek, M. M., Vandenberghe, R. R., et al. (2010) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e3181fc9aea · Cited by 83

Extensive White Matter Involvement in Patients With Frontotemporal Lobar Degeneration

Caroppo, P., Ber, I. L., Camuzat, A., Clot, F., Naccache, L., Lamari, F., de Septenville, A. L., Bertrand, A., Belliard, S., Hannequin, D., et al. (2014) · JAMA Neurology · doi:10.1001/jamaneurol.2014.1316 · Cited by 82

Expression of PC-cell-derived growth factor in benign and malignant human breast epithelium

Serrero, G. (2003) · Human Pathology · doi:10.1016/s0046-8177(03)00425-8 · Cited by 82

Voxel-Based Morphometry in Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions With and Without Progranulin Mutations

Whitwell, J. L., Jack, C. R., Baker, M., Rademakers, R., Adamson, J. L., Boeve, B. F., Knopman, D. S., Parisi, J. F., Petersen, R. C., Dickson, D. W., et al. (2007) · Archives of Neurology · doi:10.1001/archneur.64.3.371 · Cited by 82

Progranulin promotes peripheral nerve regeneration and reinnervation: role of notch signaling

Altmann, C., Vasić, V., Hardt, S., Heidler, J., Häußler, A., Wittig, I., Schmidt, M. H. H., & Tegeder, I. (2016) · Molecular Neurodegeneration · doi:10.1186/s13024-016-0132-1 · Cited by 81

Restoration of Progranulin Expression Rescues Cortical Neuron Generation in an Induced Pluripotent Stem Cell Model of Frontotemporal Dementia

Raitano, S., Ordovás, L., Muynck, L. D., Guo, W., Espuny-Camacho, I., Geraerts, M., Khurana, S., Vanuytsel, K., Tóth, B. I., Voets, T., et al. (2014) · Stem Cell Reports · doi:10.1016/j.stemcr.2014.12.001 · Cited by 80

Delayed inflammatory mRNA and protein expression after spinal cord injury

Byrnes, K. R., Washington, P. M., Knoblach, S. M., Hoffman, E. P., & Faden, A. I. (2011) · Journal of Neuroinflammation · doi:10.1186/1742-2094-8-130 · Cited by 77

Heterogeneity within a large kindred with frontotemporal dementia: A novel progranulin mutation

Bruni, A. C., Momeni, P., Bernardi, L., Tomaino, C., Frangipane, F., Elder, J., Kawarai, T., Sato, C., Pradella, S., Wakutani, Y., et al. (2007) · Neurology · doi:10.1212/01.wnl.0000265220.64396.b4 · Cited by 77

Progranulin Gene Delivery Protects Dopaminergic Neurons in a Mouse Model of Parkinson’s Disease

Van Kampen, J. M., Baranowski, D., & Kay, D. G. (2014) · PLoS ONE · doi:10.1371/journal.pone.0097032 · Cited by 76

Can Trehalose Prevent Neurodegeneration? Insights from Experimental Studies

Emanuele, E. (2014) · Current Drug Targets · doi:10.2174/1389450115666140225104705 · Cited by 75

Impaired protein degradation in FTLD and related disorders

Götzl, J. K., Lang, C. M., Haass, C., & Capell, A. (2016) · Ageing Research Reviews · doi:10.1016/j.arr.2016.04.008 · Cited by 75

The neurotrophic properties of progranulin depend on the granulin E domain but do not require sortilin binding

Muynck, L. D., Herdewyn, S., Beel, S., Scheveneels, W., Bosch, L. V. D., Robberecht, W., & Damme, P. V. (2013) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2013.04.022 · Cited by 74

The Progranulin Cleavage Products, Granulins, Exacerbate TDP-43 Toxicity and Increase TDP-43 Levels

Salazar, D. A., Butler, V. J., Argouarch, A. R., Hsu, T.-Y., Mason, A. R., Nakamura, A., McCurdy, H., Cox, D., Ng, R., Pan, G., et al. (2015) · Journal of Neuroscience · doi:10.1523/jneurosci.4808-14.2015 · Cited by 73

Structure and chromosomal location of the human granulin gene

Bhandari, V., & Bateman, A. (1992) · Biochemical and Biophysical Research Communications · doi:10.1016/0006-291x(92)92349-3 · Cited by 73

Multimodal fMRI Resting-State Functional Connectivity in Granulin Mutations: The Case of Fronto-Parietal Dementia

Premi, E., Cauda, F., Gasparotti, R., Diano, M., Archetti, S., Padovani, A., & Borroni, B. (2014) · PLoS ONE · doi:10.1371/journal.pone.0106500 · Cited by 73

Proepithelin Regulates Prostate Cancer Cell Biology by Promoting Cell Growth, Migration, and Anchorage-Independent Growth

Monamì, G., Emiliozzi, V., Bitto, A., Lovat, F., Xu, S., Goldoni, S., Fassan, M., Serrero, G., Gomella, L. G., Baffa, R., et al. (2009) · American Journal Of Pathology · doi:10.2353/ajpath.2009.080735 · Cited by 73

Brain progranulin expression in GRN-associated frontotemporal lobar degeneration

Chen‐Plotkin, A. S., Xiao, J., Geser, F., Martinez‐Lage, M., Grossman, M., Unger, T. L., Wood, E. M., Deerlin, V. M. V., Trojanowski, J. Q., & Lee, V. M. ‐. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0576-2 · Cited by 72

The Evolution of the Secreted Regulatory Protein Progranulin

Palfree, R. G. E., Bennett, H. P. J., & Bateman, A. (2015) · PLoS ONE · doi:10.1371/journal.pone.0133749 · Cited by 71

Progranulin expression is upregulated after spinal contusion in mice

Naphade, S. B., Kigerl, K. A., Jakeman, L. B., Kostyk, S. K., Popovich, P. G., & Kuret, J. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0616-y · Cited by 71

Lysophosphatidic acid and endothelin-induced proliferation of ovarian cancer cell lines is mitigated by neutralization of granulin–epithelin precursor (GEP), a prosurvival factor for ovarian cancer

Kamrava, M. R., Simpkins, F. A., Alejandro, E. U., Michener, C. M., Meltzer, E., & Kohn, E. C. (2005) · Oncogene · doi:10.1038/sj.onc.1208857 · Cited by 71

Trehalose Alters Subcellular Trafficking and the Metabolism of the Alzheimer-associated Amyloid Precursor Protein

Tien, N. T., Karaca, I., Tamboli, I. Y., & Walter, J. (2016) · Journal of Biological Chemistry · doi:10.1074/jbc.m116.719286 · Cited by 71

Increased caspase activation and decreased TDP‐43 solubility in progranulin knockout cortical cultures

Kleinberger, G., Wils, H., Ponsaerts, P., Joris, G., Timmermans, J., Broeckhoven, C. V., & Kumar‐Singh, S. (2010) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2010.06961.x · Cited by 69

Progranulin promotes Temozolomide resistance of glioblastoma by orchestrating DNA repair and tumor stemness

Bandey, I. N., Chiou, S.-H., Huang, A.-P., Tsai, J.-C., & Tu, P.-H. (2014) · Oncogene · doi:10.1038/onc.2014.92 · Cited by 69

Microglia in neurodegeneration

Hickman, S., Izzy, S., Sen, P., Morsett, L., & El Khoury, J. (2018) · Nature Neuroscience · doi:10.1038/s41593-018-0242-x · Cited by 1,710

Serum neurofilament light chain in genetic frontotemporal dementia: a longitudinal, multicentre cohort study

van der Ende, E. L., Meeter, L. H., Poos, J. M., Panman, J. L., Jiskoot, L. C., Dopper, E. G. P., Papma, J. M., de Jong, F. J., Verberk, I. M. W., Teunissen, C., et al. (2019) · The Lancet Neurology · doi:10.1016/s1474-4422(19)30354-0 · Cited by 181

Plasma glial fibrillary acidic protein is raised in progranulin-associated frontotemporal dementia

Heller, C., Foiani, M. S., Moore, K., Convery, R., Bocchetta, M., Neason, M., Cash, D. M., Thomas, D., Greaves, C. V., Woollacott, I. O., et al. (2020) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2019-321954 · Cited by 182

Fluid biomarkers in frontotemporal dementia: past, present and future

Swift, I. J., Sogorb-Esteve, A., Heller, C., Synofzik, M., Otto, M., Graff, C., Galimberti, D., Todd, E., Heslegrave, A. J., van der Ende, E. L., et al. (2021) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp-2020-323520 · Cited by 122

Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN mutations

Huang, M., Modeste, E. S., Dammer, E. B., Merino, P., Taylor, G., Duong, D. M., Deng, Q., Holler, C. J., Gearing, M., Dickson, D. W., et al. (2020) · Acta Neuropathologica Communications · doi:10.1186/s40478-020-01037-x · Cited by 102

Progranulin: normal function and role in neurodegeneration

Eriksen, J. L., & Mackenzie, I. R. A. (2008) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.04968.x · Cited by 144

Progranulin

Townley, R. A., Boeve, B. F., & Benarroch, E. E. (2017) · Neurology · doi:10.1212/wnl.0000000000004840 · Cited by 42

Progranulin as a therapeutic target in neurodegenerative diseases

Rhinn, H., Tatton, N., McCaughey, S., Kurnellas, M., & Rosenthal, A. (2022) · Trends in Pharmacological Sciences · doi:10.1016/j.tips.2021.11.015 · Cited by 208

Progranulin in neurodegenerative disease

Petkau, T. L., & Leavitt, B. R. (2014) · Trends in Neurosciences · doi:10.1016/j.tins.2014.04.003 · Cited by 174

Progranulin in neurodegenerative diseases

Harmata, B., Jędrzejowska–Szypułka, H., Pawletko, K., & Lewin‐Kowalik, J. (2018) · Postępy Higieny i Medycyny Doświadczalnej · doi:10.5604/01.3001.0012.7836