Evaluation of pharmacological induction of fatty acid β-oxidation in X-linked Adrenoleukodystrophy

The Metabolic and Molecular Bases of Inherited Disease

Scriver, R. C. (1995) · Medical Entomology and Zoology · Cited by 12,101 · see citing works

The metabolic and molecular bases of inherited disease

Chen, Y.-T. (2001) · Medical Entomology and Zoology · Cited by 6,928 · see citing works

Potent and specific inhibition of mammalian histone deacetylase both in vivo and in vitro by trichostatin A.

Yoshida, M., Kijima, M., Akita, M., & Beppu, T. (1990) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)44885-x · Cited by 1,856 · see citing works

Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters

Mosser, J., Douar, A.-M., Sarde, C.-O., Kioschis, P., Feil, R., Moser, H., Poustka, A.-M., Mandel, J.-L., & Aubourg, P. (1993) · Nature · doi:10.1038/361726a0 · Cited by 1,202 · see citing works

Trichostatin A and trapoxin: Novel chemical probes for the role of histone acetylation in chromatin structure and function

Yoshida, M., Horinouchi, S., & Beppu, T. (1995) · BioEssays · doi:10.1002/bies.950170510 · Cited by 715 · see citing works

X-linked adrenoleukodystrophy

Moser, H. W., Mahmood, A., & Raymond, G. V. (2007) · Nature Clinical Practice Neurology · doi:10.1038/ncpneuro0421 · Cited by 663 · see citing works

Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea

Steinberg, M. H., Lu, Z.-H., Barton, F. B., Terrin, M. L., Charache, S., Dover, G. J., & Hydroxyurea, T. M. S. O. (1997) · Blood · doi:10.1182/blood.v89.3.1078 · Cited by 407 · see citing works

Mechanism of action of hydroxyurea.

Yarbro, J. W. (1992) · PubMed · Cited by 399 · see citing works

Reversal of Early Neurologic and Neuroradiologic Manifestations of X-Linked Adrenoleukodystrophy by Bone Marrow Transplantation

Aubourg, P., Blanche, S., Jambaqué, I., Rocchiccioli, F., Kalifa, G., Naud-Saudreau, C., Rolland, M.-O., Debré, M., Chaussain, J.-L., Griscelli, C., et al. (1990) · New England Journal of Medicine · doi:10.1056/nejm199006283222607 · Cited by 366 · see citing works

Urea Cycle Disorders: Diagnosis, Pathophysiology, and Therapy

Brusilow, S. W., & Maestri, N. E. (1996) · Advances in Pediatrics · doi:10.1016/s0065-3101(24)00072-0 · Cited by 355 · see citing works

Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.

Singh, I., Moser, A. E., Goldfischer, S. L., & Moser, H. W. (1984) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.81.13.4203 · Cited by 348 · see citing works

Different accessibilities in chromatin to histone acetylase.

Cousens, L. S., Gallwitz, D., & Alberts, B. (1979) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)37831-6 · Cited by 340 · see citing works

Long-term effect of bone-marrow transplantation for childhood-onset cerebral X-linked adrenoleukodystrophy

Shapiro, E., Krivit, W., Lockman, L., Jambaqué, I., Peters, C., Cowan, M., Harris, R., Blanche, S., Bordigoni, P., Loes, D., et al. (2000) · The Lancet · doi:10.1016/s0140-6736(00)02629-5 · Cited by 329 · see citing works

Adrenoleukodystrophy: Incidence, new mutation rate, and results of extended family screening

Bezman, L., Moser, A. B., Raymond, G. V., Rinaldo, P., Watkins, P. A., Smith, K. D., Kass, N. E., & Moser, H. W. (2001) · Annals of Neurology · doi:10.1002/ana.101 · Cited by 324 · see citing works

A mouse model for X-linked adrenoleukodystrophy

Lu, J.-F., Lawler, A. M., Watkins, P. A., Powers, J. M., Moser, A. B., Moser, H. W., & Smith, K. D. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.17.9366 · Cited by 293 · see citing works

Long-Term Treatment of Girls with Ornithine Transcarbamylase Deficiency

Maestri, N. E., Brusilow, S. W., Clissold, D. B., & Bassett, S. S. (1996) · New England Journal of Medicine · doi:10.1056/nejm199609193351204 · Cited by 258 · see citing works

Oral sodium phenylbutyrate therapy in homozygous beta thalassemia: a clinical trial

Collins, A., Pearson, H. A., Giardina, P. J., McDonagh, K. T., Brusilow, S. W., & Dover, G. J. (1995) · Blood · doi:10.1182/blood.v85.1.43.bloodjournal85143 · Cited by 257 · see citing works

Gene redundancy and pharmacological gene therapy: Implications for X-linked adrenoleukodystrophy

Kemp, S., Wei, H.-M., Lu, J.-F., Braiterman, L. T., McGuinness, M. C., Moser, A. B., Watkins, P. A., & Smith, K. D. (1998) · Nature Medicine · doi:10.1038/3242 · Cited by 249 · see citing works

A Two-Year Trial of Oleic and Erucic Acids (“Lorenzo's Oil”) as Treatment for Adrenomyeloneuropathy

Aubourg, P., Adamsbaum, C., Lavallard-Rousseau, M.-C., Rocchiccioli, F., Cartier, N., Jambaque, I., Jakobezak, C., Lemaitre, A., Boureau, F., Wolf, C., et al. (1993) · New England Journal of Medicine · doi:10.1056/nejm199309093291101 · Cited by 242 · see citing works

Adrenoleukodystrophy: Incidence, new mutation rate, and results of extended family screening

Bezman, L., Moser, A. B., Raymond, G. V., Watkins, P. A., Smith, K. D., KASS, N. E., & Moser, H. W. (2001) · Annals of Neurology · doi:10.1002/ana.101.abs · Cited by 237 · see citing works

Adrenoleukodystrophy: impaired oxidation of very long chain fatty acids in white blood cells, cultured skin fibroblasts, and amniocytes

Singh, I., Moser, A. E., Moser, H. W., & Kishimoto, Y. (1984) · Pediatric Research · doi:10.1203/00006450-198403000-00016 · Cited by 234 · see citing works

Peroxisomal lignoceroyl-CoA ligase deficiency in childhood adrenoleukodystrophy and adrenomyeloneuropathy.

Lazo, O., Contreras, M., Hashmi, M., Stanley, W. S., Irazú, C. E., & Singh, I. (1988) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.85.20.7647 · Cited by 219 · see citing works

The future for treatment by bone marrow transplantation for adrenoleukodystrophy, metachromatic leukodystrophy, globoid cell leukodystrophy and Hurler syndrome

Krivit, W. A., Lockman, L. A., Watkins, P. A., Hirsch, J., & Shapiro, E. (1995) · Journal of Inherited Metabolic Disease · doi:10.1007/bf00710052 · Cited by 159 · see citing works

Molecular Cloning of cDNA Encoding Rat Very Long-chain Acyl-CoA Synthetase

Uchiyama, A., Aoyama, T., Kamijo, K., Uchida, Y., Kondo, N., Orii, T., & Hashimoto, T. (1996) · Journal of Biological Chemistry · doi:10.1074/jbc.271.48.30360 · Cited by 157 · see citing works

Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids

Wanders, R. J. A., van Roermund, C. W. T., van Wijland, M. J. A., Schutgens, R. B. H., van den BOSCH, H., Schram, A. W., & Tager, J. M. (1988) · Biochemical and Biophysical Research Communications · doi:10.1016/s0006-291x(88)81140-9 · Cited by 157 · see citing works

Adrenoleukodystrophy

Rizzo, W. B., Watkins, P. A., Phillips, M. W., Cranin, D., Campbell, B. D., & Avigan, J. (1986) · Neurology · doi:10.1212/wnl.36.3.357 · Cited by 153 · see citing works

Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with "Lorenzo's oil"

van Geel, B. M., Assies, J., Haverkort, E. B., Koelman, J. H. T. M., Verbeeten, B., Wanders, R. J. A., & Barth, P. G. (1999) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp.67.3.290 · Cited by 131 · see citing works

Treatment with Azacitidine of Patients with End-Stage β-Thalassemia

Lowrey, C. H., & Nienhuis, A. W. (1993) · New England Journal of Medicine · doi:10.1056/nejm199309163291205 · Cited by 123 · see citing works

The neurobiology of X-linked adrenoleukodystrophy, a demyelinating peroxisomal disorder

Dubois-Dalcq, M., Feigenbaum, V., & Aubourg, P. (1999) · Trends in Neurosciences · doi:10.1016/s0166-2236(98)01319-8 · Cited by 121 · see citing works

Mechanism of Action and Biological Profile of HMG CoA Reductase Inhibitors

Slater, E. E., & MacDonald, J. S. (1988) · Drugs · doi:10.2165/00003495-198800363-00016 · Cited by 113 · see citing works

Effects of 5-azacytidine and its 2′-deoxyderivative on cell differentiation and DNA methylation

Jones, P. A. (1985) · Pharmacology & Therapeutics · doi:10.1016/0163-7258(85)90080-4 · Cited by 110 · see citing works

FILM REVIEW

Moser, H. W. (1993) · The Lancet · doi:10.1016/0140-6736(93)90295-r · Cited by 101 · see citing works

Phosphodiesterase 5 inhibitors and nitrergic transmission—from zaprinast to sildenafil

Gibson, A. (2001) · European Journal of Pharmacology · doi:10.1016/s0014-2999(00)00824-4 · Cited by 95 · see citing works

Lovastatin and sodium phenylacetate normalize the levels of very long chain fatty acids in skin fibroblasts of X‐ adrenoleukodystrophy

Singh, I., Pahan, K., & Khan, M. (1998) · FEBS Letters · doi:10.1016/s0014-5793(98)00370-6 · Cited by 89 · see citing works

Peroxisomal fatty acid β-oxidation in HepG2 cells

Watkins, P. A., Ferrell, E. V., Pedersen, J. I., & Hoefler, G. (1991) · Archives of Biochemistry and Biophysics · doi:10.1016/0003-9861(91)90419-j · Cited by 86 · see citing works

Very long chain fatty acids in X‐linked adrenoleukodystrophy brain after treatment with Lorenzo's oil

Poulos, A., Gibson, R. A., Sharp, P. C., Beckman, K., & Grattan‐Smith, P. J. (1994) · Annals of Neurology · doi:10.1002/ana.410360509 · Cited by 72 · see citing works

Lovastatin Therapy for X-Linked Adrenoleukodystrophy: Clinical and Biochemical Observations on 12 Patients

Pai, G. S., Khan, M., Barbosa, E., Key, L. L., Craver, J. R., Curé, J. K., Betros, R., & Singh, I. (2000) · Molecular Genetics and Metabolism · doi:10.1006/mgme.2000.2977 · Cited by 70 · see citing works

Adrenoleukodystrophy: Impaired oxidation of fatty acids due to peroxisomal lignoceroyl-CoA ligase deficiency

Lazo, O., Contreras, M., Bhushan, A., Stanley, W. S., & Singh, I. (1989) · Archives of Biochemistry and Biophysics · doi:10.1016/0003-9861(89)90555-9 · Cited by 70 · see citing works

Accumulation and defective β‐oxidation of very long chain fatty acids in Zellweger's syndrome, adrenoleukodystrophy and Refsum's disease variants

Poulos, A., Singh, H., Paton, B. C., Sharp, P. C., & Derwas, N. (1986) · Clinical Genetics · doi:10.1111/j.1399-0004.1986.tb00511.x · Cited by 63 · see citing works

Brain, liver, and adipose tissue erucic and very long chain fatty acid levels in adrenoleukodystrophy patients treated with glyceryl trierucate and trioleate oils (Lorenzo's Oil)

Rasmussen, M., Moser, A. B., Borel, J. S., Khangoora, S., & Moser, H. W. (1994) · Neurochemical Research · doi:10.1007/bf00968719 · Cited by 62 · see citing works

Purification and Properties of Rat Liver Peroxisomal Very-Long-Chain Acyl-CoA Synthetase

Uchida, Y., Kondo, N., Orii, T., & Hashimoto, T. (1996) · The Journal of Biochemistry · doi:10.1093/oxfordjournals.jbchem.a021279 · Cited by 61 · see citing works

Transcriptional regulation of the human placental-like alkaline phosphatase gene and mechanisms involved in its induction by sodium butyrate.

Deng, G., Liu, G., Hu, L., Gum, J. R., & Kim, Y. S. (1992) · PubMed · Cited by 60 · see citing works

Short-chain fatty acids inhibit intestinal trefoil factor gene expression in colon cancer cells

Tran, C. P., Familari, M., Parker, L. M., Whitehead, R. H., & Giraud, A. S. (1998) · American Journal of Physiology-Gastrointestinal and Liver Physiology · doi:10.1152/ajpgi.1998.275.1.g85 · Cited by 59 · see citing works

X-Linked adrenoleukodystrophy: Defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters

Wanders, R. J. A., van Roermund, C. W. T., van Wijland, M. J. A., Nijenhuis, A. A., Tromp, A., Schutgens, R. B. H., Brouwer-Kelder, E. M., Schram, A. W., Tager, J. M., van den BOSCH, H., et al. (1987) · Clinica Chimica Acta · doi:10.1016/0009-8981(87)90177-x · Cited by 57 · see citing works

Phytanic acid alpha-oxidation in rat liver. Requirement of cytosolic factor.

Muralidharan, V. B., & Kishimoto, Y. (1984) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)90650-2 · Cited by 55 · see citing works

Two cytotoxic cell proteinase genes are differentially sensitive to sodium butyrate

Frégeau, C. J., Helgason, C. D., & Bleackley, R. C. (1992) · Nucleic Acids Research · doi:10.1093/nar/20.12.3113 · Cited by 41 · see citing works

Effects of amphetamine on urinary and tissue catecholamines in rats after inhibition of its metabolism with desmethylimipramine

Lewander, T. (1968) · European Journal of Pharmacology · doi:10.1016/0014-2999(68)90149-0 · Cited by 41 · see citing works

Retroviral Transfer and Long-Term Expression of the Adrenoleukodystrophy Gene in Human CD34 + Cells

Doerflinger, N., Micléa, J.-M., Lopez, J., Chomienne, C., Bougneres, P. F., Aubourg, P. R., & Cartier, N. (1998) · Human Gene Therapy · doi:10.1089/hum.1998.9.7-1025 · Cited by 39 · see citing works

Induction of histone acetylation and growth regulation in eryrthroleukemia cells by 4-phenylbutyrate and structural analogs.

Lea, M. A., Randolph, V. M., & Hodge, S. K. (1999) · PubMed · Cited by 38 · see citing works

Lovastatin does not correct the accumulation of very long‐chain fatty acids in tissues of adrenoleukodystrophy protein‐deficient mice

Yamada, T., Shinnoh, N., Taniwaki, T., Ohyagi, Y., Asahara, H., Horiuchi, I., & Kira, J. (2000) · Journal of Inherited Metabolic Disease · doi:10.1023/a:1005634130286 · Cited by 28 · see citing works

Simvastatin does not normalize very long chain fatty acids in adrenoleukodystrophy mice

Cartier, N., Guidoux, S., Rocchiccioli, F., & Aubourg, P. R. (2000) · FEBS Letters · doi:10.1016/s0014-5793(00)01852-4 · Cited by 26 · see citing works

CLINICAL AND HEMATOLOGICAL RESPONSES TO HYDROXYUREA IN SICILIAN PATIENTS WITH Hb S/β-THALASSEMIA

Rigano, P., Rodgers, G. P., Renda, D., Renda, M. C., Aquino, A., & Maggio, A. (2001) · Hemoglobin · doi:10.1081/hem-100103065 · Cited by 23 · see citing works

Transcriptional upregulation of TGF-α by phenylacetate and phenylbutyrate is associated with differentiation of human melanoma cells

Liu, L., Hudgins, R. W., Miller, A. C., Chen, L.-C., & Samid, D. (1995) · Cytokine · doi:10.1006/cyto.1995.0061 · Cited by 23 · see citing works

Molecular and functional characterization of the murine glucocerebrosidase gene

Carstea, E. D., Murray, G. J., & O’Neill, R. (1992) · Biochemical and Biophysical Research Communications · doi:10.1016/s0006-291x(05)80049-x · Cited by 15 · see citing works

Untitled work

Cann, S. A. H., Netten, J. P. V., & Netten, C. V. (2000) · Pure Amsterdam UMC · doi:10.1002/1531-8249(200003)47:3

Inhibition of apolipoprotein B100 secretion by lipid-induced hepatic endoplasmic reticulum stress in rodents

Ota, T., Gayet, C., & Ginsberg, H. N. (2008) · Journal of Clinical Investigation · doi:10.1172/jci32752 · Cited by 376 · see citing works

Peroxisomal disorders: The single peroxisomal enzyme deficiencies

Wanders, R. J. A., & Waterham, H. R. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2006.08.010 · Cited by 279 · see citing works

Adrenoleukodystrophy—Neuroendocrine pathogenesis and redefinition of natural history

Kemp, S., Huffnagel, I. C., Linthorst, G. E., Wanders, R. J., & Engelen, M. (2016) · Nature Reviews Endocrinology · doi:10.1038/nrendo.2016.90 · Cited by 275 · see citing works

X-linked adrenoleukodystrophy: Clinical, biochemical and pathogenetic aspects

Berger, J., & Gärtner, J. (2006) · Biochimica et Biophysica Acta (BBA) - Molecular Cell Research · doi:10.1016/j.bbamcr.2006.07.010 · Cited by 199 · see citing works

The role of ELOVL1 in very long-chain fatty acid homeostasis and X-linked adrenoleukodystrophy

Ofman, R., Dijkstra, I. M. E., van Roermund, C. W. T., Burger, N., Turkenburg, M., van Cruchten, A., van Engen, C. E., Wanders, R. J. A., & Kemp, S. (2010) · EMBO Molecular Medicine · doi:10.1002/emmm.201000061 · Cited by 170 · see citing works

Histone deacetylase inhibitors: possible implications for neurodegenerative disorders

Hahnen, E., Hauke, J., Tränkle, C., Eyüpoglu, I. Y., Wirth, B., & Blümcke, I. (2008) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.17.2.169 · Cited by 164 · see citing works

ABC Multidrug Transporters: Target for Modulation of Drug Pharmacokinetics and Drug-Drug Interactions

Marquez, B., & Françoise Van Bambeke, A. (2011) · Current Drug Targets · doi:10.2174/138945011795378504 · Cited by 165 · see citing works

PEX11α Is Required for Peroxisome Proliferation in Response to 4-Phenylbutyrate but Is Dispensable for Peroxisome Proliferator-Activated Receptor Alpha-Mediated Peroxisome Proliferation

Li, X., Baumgart‐Vogt, E., Dong, G.-X., Morrell, J. C., Jiménez‐Sánchez, G., Valle, D. L., Smith, K. D., & Gould, S. J. (2002) · Molecular and Cellular Biology · doi:10.1128/mcb.22.23.8226-8240.2002 · Cited by 157 · see citing works

Function of prokaryotic and eukaryotic ABC proteins in lipid transport

Pohl, A., Devaux, P. F., & Herrmann, A. (2005) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2004.12.007 · Cited by 149 · see citing works

Role of ALDP (ABCD1) and Mitochondria in X-Linked Adrenoleukodystrophy

McGuinness, M. C., Lu, J.-F., Zhang, H.-P., Dong, G.-X., Heinzer, A. K., Watkins, P. A., Powers, J., & Smith, K. D. (2003) · Molecular and Cellular Biology · doi:10.1128/mcb.23.2.744-753.2003 · Cited by 147 · see citing works

General aspects and neuropathology of X-linked adrenoleukodystrophy

Ferrer, I., Aubourg, P., & Pujol, A. (2010) · Brain Pathology · doi:10.1111/j.1750-3639.2010.00390.x · Cited by 139 · see citing works

Peroxisomal ABC transporters

Theodoulou, F. L., Holdsworth, M. J., & Baker, A. (2006) · FEBS Letters · doi:10.1016/j.febslet.2005.12.095 · Cited by 119 · see citing works

Biochemical aspects of X-linked adrenoleukodystrophy

Kemp, S., & Wanders, R. (2010) · Brain Pathology · doi:10.1111/j.1750-3639.2010.00391.x · Cited by 116 · see citing works

The peroxisomal ABC transporter family

Wanders, R. J. A., Visser, W. F., van Roermund, C. W. T., Kemp, S., & Waterham, H. R. (2006) · Pflügers Archiv - European Journal of Physiology · doi:10.1007/s00424-006-0142-x · Cited by 111 · see citing works

A Novel Fluorescent Sensor Protein for Visualization of Redox States in the Cytoplasm and in Peroxisomes

Yano, T., Oku, M., Akeyama, N., Itoyama, A., Yurimoto, H., Kuge, S., Fujiki, Y., & Sakai, Y. (2010) · Molecular and Cellular Biology · doi:10.1128/mcb.00121-10 · Cited by 110 · see citing works

Elongation of very long-chain fatty acids is enhanced in X-linked adrenoleukodystrophy

Kemp, S., Valianpour, F., Denis, S., Ofman, R., Sanders, R.-J., Mooyer, P., Barth, P. G., & Wanders, R. J. A. (2005) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2004.09.015 · Cited by 97 · see citing works

Current and Future Pharmacological Treatment Strategies in X‐Linked Adrenoleukodystrophy

Berger, J., Pujol, A., Aubourg, P. R., & Forss‐Petter, S. (2010) · Brain Pathology · doi:10.1111/j.1750-3639.2010.00393.x · Cited by 90 · see citing works

A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-linked adrenoleukodystrophy

Heinzer, A. K. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg126 · Cited by 87 · see citing works

X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism, ABC half-transporters and the complicated route to treatment

Kemp, S., & Wanders, R. J. A. (2007) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2006.10.001 · Cited by 82 · see citing works

Peroxisomal ABC Transporters: An Update

Tawbeh, A., Gondcaille, C., Trompier, D., & Savary, S. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22116093 · Cited by 68 · see citing works

Individual histone deacetylases in Drosophila modulate transcription of distinct genes

Cho, Y., Griswold, A., Campbell, C., & Min, K.-T. (2005) · Genomics · doi:10.1016/j.ygeno.2005.07.007 · Cited by 71 · see citing works

A Thyroid Hormone–Based Strategy for Correcting the Biochemical Abnormality in X-Linked Adrenoleukodystrophy

Hartley, M. D., Kirkemo, L. L., Banerji, T., & Scanlan, T. S. (2017) · Endocrinology · doi:10.1210/en.2016-1842 · Cited by 71 · see citing works

Phenylbutyrate up-regulates the adrenoleukodystrophy-related gene as a nonclassical peroxisome proliferator

Gondcaille, C., Depreter, M., Fourcade, S., Lecca, M. R., Leclercq, S., Martin, P. G. P., Pineau, T., Cadepond, F., El‐Etr, M., Bertrand, N., et al. (2005) · The Journal of Cell Biology · doi:10.1083/jcb.200501036 · Cited by 70 · see citing works

Lipid-regulating properties of butyric acid and 4-phenylbutyric acid: Molecular mechanisms and therapeutic applications

He, B., & Moreau, R. F. (2019) · Pharmacological Research · doi:10.1016/j.phrs.2019.04.002 · Cited by 60 · see citing works

Activation of the stress proteome as a mechanism for small molecule therapeutics

Brose, R. D., Shin, G., McGuinness, M. C., Schneidereith, T., Purvis, S. H., Dong, G.-X., Keefer, J. R., Spencer, F. A., & Smith, K. D. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds247 · Cited by 46 · see citing works

Enigma, a mitochondrial protein affecting lifespan and oxidative stress response in Drosophila

Mourikis, P., Hurlbut, G. D., & Artavanis‐Tsakonas, S. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0510564103 · Cited by 41 · see citing works

HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes

Singh, J., Khan, M., & Singh, I. (2011) · Journal of Lipid Research · doi:10.1194/jlr.m017491 · Cited by 37 · see citing works

Emerging chemotherapeutic strategies for Huntington’s disease

Ryu, H., & Ferrante, R. J. (2005) · Expert Opinion on Emerging Drugs · doi:10.1517/14728214.10.2.345 · Cited by 29 · see citing works

Baicalein 5,6,7‐trimethyl ether, a flavonoid derivative, stimulates fatty acid β‐oxidation in skin fibroblasts of X‐linked adrenoleukodystrophy

Morita, M., Takahashi, I., Kanai, M., Okafuji, F., Iwashima, M., Hayashi, T., Watanabe, S., Hamazaki, T., Shimozawa, N., Suzuki, Y., et al. (2004) · FEBS Letters · doi:10.1016/j.febslet.2004.11.102 · Cited by 25 · see citing works

Histone Deacetylase Inhibitor Upregulates Peroxisomal Fatty Acid Oxidation and Inhibits Apoptotic Cell Death in Abcd1-Deficient Glial Cells

Singh, J., Khan, M., Pujol, A., Baarine, M., & Singh, I. (2013) · PLoS ONE · doi:10.1371/journal.pone.0070712 · Cited by 24 · see citing works

Mouse Models and Genetic Modifiers in X-Linked Adrenoleukodystrophy

Heinzer, A. K., McGuinness, M. C., Lu, J., Stine, O. C., Wei, H., Vlies, M. V. D., Dong, G.-X., Powers, J. F., Watkins, P. A., & Smith, K. D. (2003) · Advances in experimental medicine and biology · doi:10.1007/978-1-4419-9072-3_12 · Cited by 23 · see citing works

Caffeic acid phenethyl ester induces adrenoleukodystrophy (Abcd2) gene in human X-ALD fibroblasts and inhibits the proinflammatory response in Abcd1/2 silenced mouse primary astrocytes

Singh, J., Khan, M., & Singh, I. (2013) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2013.01.004 · Cited by 21 · see citing works

X-Linked Adrenoleukodystrophy

(2006) · Springer eBooks · doi:10.1007/3-540-29623-9_9160 · Cited by 21 · see citing works

Evaluation of Therapy of X-Linked Adrenoleukodystrophy

Moser, H. W., Fatemi, A. S., Zackowski, K. M., Smith, S., Golay, X., Muenz, L. R., & Raymond, G. V. (2004) · Neurochemical Research · doi:10.1023/b:nere.0000021245.12181.90 · Cited by 17 · see citing works

Method for Measurement of Peroxisomal Very Long-Chain Fatty Acid Beta-Oxidation and De Novo C26:0 Synthesis Activity in Living Cells Using Stable-Isotope Labeled Docosanoic Acid

van de Beek, M.-C., Dijkstra, I. M. E., & Kemp, S. (2017) · Methods in molecular biology · doi:10.1007/978-1-4939-6937-1_5 · Cited by 16 · see citing works

X-linked Adrenoleukodystrophy

Trompier, D., & Savary, S. (2013) · Colloquium Series on The Genetic Basis of Human Disease · doi:10.4199/c00075ed1v01y201303gbd004 · Cited by 16 · see citing works

Activity of the yeast Tat2p tryptophan permease is sensitive to the anti-tumor agent 4-phenylbutyrate

Liu, M., Brusilow, W. S. A., & Needleman, R. B. (2004) · Current Genetics · doi:10.1007/s00294-004-0531-7 · Cited by 14 · see citing works

Treatment: Present Status and New Trends

Walter, J. H., & Wraith, J. E. (2006) · Springer eBooks · doi:10.1007/978-3-540-28785-8_5 · Cited by 12 · see citing works

Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy

Bougnères, P., & Le Stunff, C. (2025) · Genes · doi:10.3390/genes16050590 · Cited by 11 · see citing works

Treatment of an adrenomyeloneuropathy patient with Lorenzo's oil and supplementation with docosahexaenoic acid-A case report

Terre’Blanche, G., der Walt, M. M. V., Bergh, J. J., & Mienie, L. J. (2011) · Lipids in Health and Disease · doi:10.1186/1476-511x-10-152 · Cited by 11 · see citing works

Biochemical effect of intravenous arginine butyrate in x-linked adrenoleukodystrophy

McGovern, M. M., Wasserstein, M., Aron, A. M., & Perrine, S. P. (2003) · The Journal of Pediatrics · doi:10.1067/mpd.2003.201 · Cited by 10 · see citing works

A sensitive pre-column derivatization method for the analysis of free fatty acids by RP-HPLC with fluorescence detector and its application to Caragana species

Zeng, Z., Ji, Z., Hu, N., Bai, B., Wang, H., & Suo, Y. (2017) · Journal of Chromatography B · doi:10.1016/j.jchromb.2017.07.037 · Cited by 9 · see citing works

Arginase induction by sodium phenylbutyrate in mouse tissues and human cell lines

Kern, R. M., Yang, Z., Kim, P. S., Grody, W. W., Iyer, R. K., & Cederbaum, S. D. (2006) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2006.07.002 · Cited by 8 · see citing works

Transcriptional changes associated with recovery from heart failure in the SHR

Brooks, W. W., Shen, S., Conrad, C. H., Goldstein, R. H., Deng, L. L., & Bing, O. H. L. (2010) · Journal of Molecular and Cellular Cardiology · doi:10.1016/j.yjmcc.2010.06.002 · Cited by 6 · see citing works

The mouse as a model to understand peroxisomal biogenesis and its disorders

Brites, P., Wanders, R. J. A., & Waterham, H. R. (2004) · Drug Discovery Today Disease Models · doi:10.1016/j.ddmod.2004.09.003 · Cited by 6 · see citing works

Adrenoleukodystrophies

Moser, H. W. (2004) · Elsevier eBooks · doi:10.1016/b978-012439510-7/50087-5 · Cited by 4 · see citing works

Therapeutic Strategies for X-Linked Adrenoleukodystrophy, a Representative Peroxisomal Disorder

Morita, M. (2019) · Springer eBooks · doi:10.1007/978-981-15-1169-1_8 · Cited by 2 · see citing works

Leukodystrophies

Garbern, J. Y. (2005) · Neurological disease and therapy · doi:10.1201/9780849358364.ch20 · Cited by 1 · see citing works

The metabolic profile of phenylbutyric acid and its antioxidant capacity in vervet monkeys

Linde, V. D. R. R., & Johanna, W. (2010)

NRF2 and RIP140 as new therapeutic targets for X-linked adrenoleukodystrophy (X-ALD): Control of redox/metabolic homeostasis and inflammation

Ranea‐Robles, P. (2017) · Tesis Doctorals en Xarxa (Consorci de Serveis Universitaris de Catalunya)

Chapter 17 X-Linked Spastic Paraplegia

Garbern, J. Y. (2007) · Blue books of neurology · doi:10.1016/s1877-184x(09)70091-7

Role of ALDP (ABCD1) and Mitochondria in X-Linked Adrenoleukodystrophy

McGuinness, M. C., Lu, J.-F., Zhang, H.-P., Dong, G.-X., Heinzer, A. K., Watkins, P. A., Powers, J., & Smith, K. D. (2003) · Molecular and Cellular Biology · doi:10.1128/mcb.23.2.744-753.2003 · Cited by 147 · see citing works

Biochemical aspects of X-linked adrenoleukodystrophy

Kemp, S., & Wanders, R. (2010) · Brain Pathology · doi:10.1111/j.1750-3639.2010.00391.x · Cited by 116 · see citing works

Very-Long-Chain Fatty Acid Metabolism in Adrenoleukodystrophy Protein-Deficient Mice

Yamada, T., Shinnoh, N., Kondo, A., Uchiyama, A., Shimozawa, N., Kira, J., & Kobayashi, T. (2000) · Cell Biochemistry and Biophysics · doi:10.1385/cbb:32:1-3:239 · Cited by 14 · see citing works

Cholesterol-deprivation increases mono-unsaturated very long-chain fatty acids in skin fibroblasts from patients with X-linked adrenoleukodystrophy

Engelen, M., Ofman, R., Mooijer, P. A. W., Poll‐The, B. T., Wanders, R. J. A., & Kemp, S. (2008) · Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids · doi:10.1016/j.bbalip.2007.12.003 · Cited by 21 · see citing works

Bezafibrate lowers very long‐chain fatty acids in X‐linked adrenoleukodystrophy fibroblasts by inhibiting fatty acid elongation

Engelen, M., Schackmann, M. J. A., Ofman, R., Sanders, R., Dijkstra, I. M. E., Houten, S. M., Fourcade, S., Pujol, A., Poll‐The, B. T., Wanders, R. J. A., et al. (2012) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-012-9471-4 · Cited by 49 · see citing works