Telomerase and idiopathic pulmonary fibrosis
Open accessAbstract
Idiopathic pulmonary fibrosis (IPF) is the most common manifestation of telomere-mediated disorders. Germline mutations in the essential telomerase genes, hTERT and hTR, are the causal genetic defect in up to one-sixth of pulmonary fibrosis families. The presence of telomerase mutations in this subset is significant for clinical decisions as affected individuals can develop extra-pulmonary complications related to telomere shortening such as bone marrow failure and cryptogenic liver cirrhosis. There is also evidence that IPF is an ancestral manifestation of autosomal dominant telomere syndromes where, with successive generations, the disease evolves from pulmonary fibrosis into a bone marrow failure-predominant disorder, defining a unique form of genetic anticipation. Here I review the significance of telomere defects for understanding the genetics, disease patterns and pathophysiology of IPF. The importance of this diagnosis for patient care decisions will also be discussed.
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Idiopathic pulmonary fibrosis is the most frequent disease manifestation caused by telomere defects.
“Among these, the lung disease idiopathic pulmonary fibrosis (IPF) — best known until recently by its ‘idiopathic’ adjective — is the most common manifestation of telomere-mediated disease12.”
From The telomere syndromes (2012) · cites this paper as [12]
If slow-turnover tissues need several extra hits before phenotypes appear, this could account for telomere-related lung disease being mild, adult-onset, and usually diagnosed after midlife.
“A model that requires multiple additional hits for phenotypes to manifest in slow-turnover tissues could explain why telomere-mediated lung disease represents an attenuated, adult-onset phenotype that is commonly seen after middle age12,99.”
From The telomere syndromes (2012) · cites this paper as [12]
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(2002) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.165.2.ats01 · Cited by 4,196 · see citing works
Identification of a specific telomere terminal transferase activity in tetrahymena extracts
Greider, C. W., & Blackburn, E. H. (1985) · Cell · doi:10.1016/0092-8674(85)90170-9 · Cited by 3,400 · see citing works
Telomere Shortening and Tumor Formation by Mouse Cells Lacking Telomerase RNA
Blasco, M. A., Lee, H.-W., Hande, M. P., Samper, E., Lansdorp, P. M., DePinho, R. A., & Greider, C. W. (1997) · Cell · doi:10.1016/s0092-8674(01)80006-4 · Cited by 2,108 · see citing works
A telomeric sequence in the RNA of Tetrahymena telomerase required for telomere repeat synthesis
Greider, C. W., & Blackburn, E. H. (1989) · Nature · doi:10.1038/337331a0 · Cited by 1,643 · see citing works
Telomerase Mutations in Families with Idiopathic Pulmonary Fibrosis
Armanios, M. Y., Chen, J. J.-L., Cogan, J. D., Alder, J. K., Ingersoll, R. G., Markin, C., Lawson, W. E., Xie, M., Vulto, I., Phillips, J. A., et al. (2007) · New England Journal of Medicine · doi:10.1056/nejmoa066157 · Cited by 1,357 · see citing works
Self-citationLongevity, Stress Response, and Cancer in Aging Telomerase-Deficient Mice
Rudolph, K. L., Chang, S., Lee, H.-W., Blasco, M., Gottlieb, G. J., Greider, C., & DePinho, R. A. (1999) · Cell · doi:10.1016/s0092-8674(00)80580-2 · Cited by 1,349 · cited 1× · see citing works
“However, after breeding for successive generations, mice that have short telomeres developed degenerative disease that was most evident in tissues of high turnover[19, 20].”
BackgroundIncidence and Prevalence of Idiopathic Pulmonary Fibrosis
Raghu, G., Weycker, D., Edelsberg, J., Bradford, W. Z., & Oster, G. (2006) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200602-163oc · Cited by 1,310 · see citing works
Essential role of mouse telomerase in highly proliferative organs
Lee, H.-W., Blasco, M. A., Gottlieb, G. J., Horner, J. W., Greider, C. W., & DePinho, R. A. (1998) · Nature · doi:10.1038/33345 · Cited by 1,270 · cited 3× · see citing works
“The consequences of telomerase deficiency were studied in model organisms before telomerase mutations were discovered in the setting of human disease[18, 19].”
BackgroundA telomerase component is defective in the human disease dyskeratosis congenita
Mitchell, J. R., Wood, E., & Collins, K. (1999) · Nature · doi:10.1038/990141 · Cited by 1,168 · see citing works
Greider, C. W., & Blackburn, E. H. (1987) · Cell · doi:10.1016/0092-8674(87)90576-9 · Cited by 1,150 · see citing works
Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity
Cottin, V., Nunès, H., Brillet, P., Delaval, P.-E., Devouassoux, G., Tillie‐Leblond, I., Israël‐Biet, D., Court‐Fortune, I., Valeyre, D., & Cordier, J.-F. (2005) · European Respiratory Journal · doi:10.1183/09031936.05.00021005 · Cited by 1,001 · see citing works
Heiss, N. S., Knight, S. W., Vulliamy, T. J., Klauck, S. M., Wiemann, S., Mason, P. J., Poustka, A., & Dokal, I. (1998) · Nature Genetics · doi:10.1038/ng0598-32 · Cited by 976 · see citing works
The RNA component of telomerase is mutated in autosomal dominant dyskeratosis congenita
Vulliamy, T., Marrone, A., Goldman, F., Dearlove, A., Bessler, M., Mason, P. J., & Dokal, I. (2001) · Nature · doi:10.1038/35096585 · Cited by 960 · see citing works
Adult-onset pulmonary fibrosis caused by mutations in telomerase
Tsakiri, K. D., Cronkhite, J. T., Kuan, P. J., Xing, C., Raghu, G., Weissler, J. C., Rosenblatt, R. L., Shay, J. W., & Garcia, C. K. (2007) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0701009104 · Cited by 894 · cited 2× · see citing works
“Among these, IPF/usual interstitial pneumonia is most common seen in approximately 65% of cases[37, 38, 40, 42, 43].”
BackgroundShort telomeres are a risk factor for idiopathic pulmonary fibrosis
Alder, J. K., Chen, J. J.-L., Lancaster, L., Danoff, S., Su, S.-C., Cogan, J. D., Vulto, I., Xie, M., Qi, X., Tuder, R. M., et al. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0804280105 · Cited by 805 · see citing works
Self-citationMutations in <i>TERT,</i> the Gene for Telomerase Reverse Transcriptase, in Aplastic Anemia
Yamaguchi, H., Calado, R. T., Ly, H., Kajigaya, S., Baerlocher, G. M., Chanock, S. J., Lansdorp, P. M., & Young, N. S. (2005) · New England Journal of Medicine · doi:10.1056/nejmoa042980 · Cited by 723 · see citing works
Lung Volumes and Emphysema in Smokers with Interstitial Lung Abnormalities
Washko, G. R., Hunninghake, G. M., Fernandez, I. E., Nishino, M., Okajima, Y., Yamashiro, T., Ross, J. C., Estépar, R. S. J., Lynch, D. A., Brehm, J. M., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1007285 · Cited by 613 · see citing works
Secondary Structure of Vertebrate Telomerase RNA
Chen, J.-L., Blasco, M. A., & Greider, C. W. (2000) · Cell · doi:10.1016/s0092-8674(00)80687-x · Cited by 605 · see citing works
Dyskeratosis congenita in all its forms
Dokal, I. (2000) · British Journal of Haematology · doi:10.1046/j.1365-2141.2000.02109.x · Cited by 575 · see citing works
Telomere Shortening in Familial and Sporadic Pulmonary Fibrosis
Cronkhite, J. T., Xing, C., Raghu, G., Chin, K. M., Torres, F., Rosenblatt, R. L., & Kim Garcia, C. (2008) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200804-550oc · Cited by 573 · see citing works
A Box H/ACA Small Nucleolar RNA-Like Domain at the Human Telomerase RNA 3′ End
Mitchell, J. R., Cheng, J., & Collins, K. (1999) · Molecular and Cellular Biology · doi:10.1128/mcb.19.1.567 · Cited by 510 · see citing works
Mechanisms of trinucleotide repeat instability during human development
McMurray, C. T. (2010) · Nature Reviews Genetics · doi:10.1038/nrg2828 · Cited by 496 · see citing works
Vulliamy, T., Marrone, A., Szydlo, R., Walne, A., Mason, P. J., & Dokal, I. (2004) · Nature Genetics · doi:10.1038/ng1346 · Cited by 461 · see citing works
Armanios, M., Chen, J.-L., Chang, Y.-P. C., Brodsky, R. A., Hawkins, A., Griffin, C. A., Eshleman, J. R., Cohen, A. R., Chakravarti, A., Hamosh, A., et al. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0508124102 · Cited by 452 · see citing works
Self-citationClinical and Pathologic Features of Familial Interstitial Pneumonia
Steele, M. P., Speer, M. C., Loyd, J. E., Brown, K. K., Herron, A., Slifer, S. H., Burch, L. H., Wahidi, M. M., Phillips, J. A., Sporn, T. A., et al. (2005) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200408-1104oc · Cited by 432 · see citing works
Savage, S. A., Giri, N., Baerlocher, G. M., Orr, N., Lansdorp, P. M., & Alter, B. P. (2008) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2007.10.004 · Cited by 414 · see citing works
Telomere Lengths, Pulmonary Fibrosis and Telomerase (TERT) Mutations
Diaz de Leon, A., Cronkhite, J. T., Katzenstein, A.-L. A., Godwin, J. D., Raghu, G., Glazer, C. S., Rosenblatt, R. L., Girod, C. E., Garrity, E. R., Xing, C., et al. (2010) · PLoS ONE · doi:10.1371/journal.pone.0010680 · Cited by 377 · see citing works
Syndromes of Telomere Shortening
Armanios, M. (2009) · Annual Review of Genomics and Human Genetics · doi:10.1146/annurev-genom-082908-150046 · Cited by 317 · see citing works
Self-citationWalne, A. J., Vulliamy, T., Beswick, R., Kirwan, M., & Dokal, I. (2008) · Blood · doi:10.1182/blood-2008-05-153445 · Cited by 317 · see citing works
Short Telomeres, even in the Presence of Telomerase, Limit Tissue Renewal Capacity
Hao, L.-Y., Armanios, M., Strong, M. A., Karim, B., Feldser, D. M., Huso, D., & Greider, C. W. (2005) · Cell · doi:10.1016/j.cell.2005.11.020 · Cited by 302 · see citing works
Self-citationMutations of the human telomerase RNA gene (TERC) in aplastic anemia and myelodysplastic syndrome
Yamaguchi, H., Baerlocher, G. M., Lansdorp, P. M., Chanock, S. J., Nunez, O., Sloand, E., & Young, N. S. (2003) · Blood · doi:10.1182/blood-2003-01-0335 · Cited by 301 · see citing works
Telomere Length Is a Determinant of Emphysema Susceptibility
Alder, J. K., Guo, N., Kembou, F., Parry, E. M., Anderson, C. J., Gorgy, A. I., Walsh, M. F., Sussan, T., Biswal, S., Mitzner, W., et al. (2011) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201103-0520oc · Cited by 265 · see citing works
Self-citationShort Telomeres are Sufficient to Cause the Degenerative Defects Associated with Aging
Armanios, M., Alder, J. K., Parry, E. M., Karim, B., Strong, M. A., & Greider, C. W. (2009) · The American Journal of Human Genetics · doi:10.1016/j.ajhg.2009.10.028 · Cited by 257 · see citing works
Self-citationA Spectrum of Severe Familial Liver Disorders Associate with Telomerase Mutations
Calado, R. T., Regal, J. A., Kleiner, D. E., Schrump, D. S., Peterson, N. R., Pons, V., Chanock, S. J., Lansdorp, P. M., & Young, N. S. (2009) · PLoS ONE · doi:10.1371/journal.pone.0007926 · Cited by 252 · see citing works
The telomerase reverse transcriptase is limiting and necessary for telomerase function in vivo
Liu, Y., Snow, B. E., Hande, M. P., Yeung, D., Erdmann, N. J., Wakeham, A., Itie, A., Siderovski, D. P., Lansdorp, P. M., Robinson, M. O., et al. (2000) · Current Biology · doi:10.1016/s0960-9822(00)00805-8 · Cited by 244 · see citing works
Dyskeratosis congenita: a genetic disorder of many faces
Kirwan, M., & Dokal, I. S. (2007) · Clinical Genetics · doi:10.1111/j.1399-0004.2007.00923.x · Cited by 238 · see citing works
Parry, E. M., Alder, J. K., Qi, X., Chen, J. J.-L., & Armanios, M. (2011) · Blood · doi:10.1182/blood-2010-11-322149 · Cited by 194 · see citing works
Self-citationTuder, R. M., Yoshida, T., Arap, W., Pasqualini, R., & Petrache, I. (2006) · Proceedings of the American Thoracic Society · doi:10.1513/pats.200603-054ms · Cited by 174 · see citing works
Du, H.-Y., Pumbo, E., Ivanovich, J., An, P., Maziarz, R. T., Reiss, U. M., Chirnomas, D., Shimamura, A., Vlachos, A., Lipton, J. M., et al. (2009) · Blood · doi:10.1182/blood-2008-07-166421 · Cited by 158 · see citing works
Dietz, A. C., Orchard, P. J., Baker, K. S., Giller, R., Savage, S. A., Alter, B. P., & Tolar, J. (2010) · Bone Marrow Transplantation · doi:10.1038/bmt.2010.65 · Cited by 151 · see citing works
Telomerase RNA structure and function: implications for dyskeratosis congenita
Chen, J.-L., & Greider, C. W. (2004) · Trends in Biochemical Sciences · doi:10.1016/j.tibs.2004.02.003 · Cited by 146 · see citing works
It all comes together at the ends: Telomerase structure, function, and biogenesis
Podlevsky, J. D., & Chen, J. J. ‐. (2011) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/j.mrfmmm.2011.11.002 · Cited by 144 · see citing works
Genetics of leukocyte telomere length and its role in atherosclerosis
Aviv, A. (2012) · Mutation Research - Fundamental and Molecular Mechanisms of Mutagenesis · doi:10.1016/j.mrfmmm.2011.05.001 · Cited by 143 · see citing works
Dyskeratosis congenita as a disorder of telomere maintenance
Nelson, N. D., & Bertuch, A. A. (2011) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/j.mrfmmm.2011.06.008 · Cited by 138 · see citing works
Strong, M. A., Vidal-Cardenas, S. L., Karim, B. O., Yu, H. M., Guo, N., & Greider, C. W. (2011) · Molecular and Cellular Biology · doi:10.1128/mcb.05312-11 · Cited by 134 · see citing works
Gribbin, J., Hubbard, R., & Smith, C. (2009) · Respiratory Medicine · doi:10.1016/j.rmed.2008.11.001 · Cited by 132 · see citing works
De La Fuente, J., & Dokal, I. (2007) · Pediatric Transplantation · doi:10.1111/j.1399-3046.2007.00721.x · Cited by 119 · see citing works
Alder, J. K., Cogan, J. D., Brown, A. F., Anderson, C. J., Lawson, W. E., Lansdorp, P. M., Phillips, J. A., Loyd, J. E., Chen, J. J.-L., & Armanios, M. (2011) · PLoS Genetics · doi:10.1371/journal.pgen.1001352 · Cited by 119 · see citing works
Self-citationKirwan, M., Vulliamy, T., Marrone, A., Walne, A. J., Beswick, R., Hillmen, P., Kelly, R., Stewart, A., Bowen, D., Schonland, S. O., et al. (2009) · Human Mutation · doi:10.1002/humu.21115 · Cited by 109 · cited 1× · see citing works
“In addition, myelodysplastic syndromes (MDS), and acute myeloid leukemia (AML) occur more frequently in IPF families[42, 43, 53].”
BackgroundShort Telomeres Compromise β-Cell Signaling and Survival
Guo, N., Parry, E. M., Li, L.-S., Kembou, F., Lauder, N., Hussain, M. A., Berggren, P.-O., & Armanios, M. (2011) · PLoS ONE · doi:10.1371/journal.pone.0017858 · Cited by 100 · see citing works
Self-citationRisk factors for idiopathic pulmonary fibrosis in a Mexican population. A case-control study
Figueroa, M. C. G.-S., Carrillo, G., Pérez‐Padilla, R., Fernández-Plata, M. R., Buendía-Roldán, I., Vargas, M. H., & Selman, M. (2009) · Respiratory Medicine · doi:10.1016/j.rmed.2009.08.013 · Cited by 92 · see citing works
Pulmonary fibrosis in families.
Loyd, J. E. (2003) · PubMed · Cited by 89 · see citing works
Dyskeratosis congenita: The first NIH clinical research workshop
Savage, S. A., Dokal, I. S., Armanios, M. Y., Aubert, G., Cowen, E. W., Domingo, D. L., Giri, N., Greene, M. H., Orchard, P. J., Tolar, J., et al. (2009) · Pediatric Blood & Cancer · doi:10.1002/pbc.22061 · Cited by 69 · see citing works
Self-citationDecreased dyskerin levels as a mechanism of telomere shortening in X-linked dyskeratosis congenita
Parry, E. M., Alder, J. K., Lee, S. S., Phillips, J. A., Loyd, J. E., Duggal, P., & Armanios, M. Y. (2011) · Journal of Medical Genetics · doi:10.1136/jmg.2010.085100 · Cited by 66 · see citing works
Self-citationDror, Y., Freedman, M. H., Leaker, M., Verbeek, J., Armstrong, C. A., Saunders, F., & Doyle, J. (2003) · Bone Marrow Transplantation · doi:10.1038/sj.bmt.1703931 · Cited by 63 · see citing works
Update in Lung Transplantation 2008
Lama, V. N. (2009) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200902-0177up · Cited by 27 · see citing works
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Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline
Raghu, G., Remy-Jardin, M., Myers, J. L., Richeldi, L., Ryerson, C. J., Lederer, D. J., Behr, J., Cottin, V., Danoff, S. K., Morell, F., et al. (2018) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201807-1255st · Cited by 4,103 · see citing works
Armanios, M., & Blackburn, E. H. (2012) · Nature Reviews Genetics · doi:10.1038/nrg3246 · Cited by 982 · see citing works
Self-citationTelomeres and telomerase: three decades of progress
Shay, J. W., & Wright, W. E. (2019) · Nature Reviews Genetics · doi:10.1038/s41576-019-0099-1 · Cited by 924 · see citing works
Shared and distinct mechanisms of fibrosis
Distler, J. H. W., Györfi, A., Ramanujam, M., Whitfield, M. L., Königshoff, M., & Lafyatis, R. (2019) · Nature Reviews Rheumatology · doi:10.1038/s41584-019-0322-7 · Cited by 645 · see citing works
A three-dimensional model of human lung development and disease from pluripotent stem cells
Chen, Y., Huang, S. X. L., de Carvalho, A. L. R. T., Ho, S., Islam, M. N., Volpi, S., Notarangelo, L. D., Ciancanelli, M. J., Casanova, J., Bhattacharya, J., et al. (2017) · Nature Cell Biology · doi:10.1038/ncb3510 · Cited by 606 · see citing works
Oxidative stress and pulmonary fibrosis
Cheresh, P., Kim, S.-J., Tulasiram, S., & Kamp, D. W. (2012) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2012.11.021 · Cited by 527 · see citing works
Targeting PI3K/AKT signaling for treatment of idiopathic pulmonary fibrosis
Wang, J., Hu, K., Cai, X., Yang, B., He, Q., Wang, J., & Weng, Q. (2021) · Acta Pharmaceutica Sinica B · doi:10.1016/j.apsb.2021.07.023 · Cited by 464 · see citing works
Cai, Y., Song, W., Li, J., Jing, Y., Liang, C., Zhang, L., Zhang, X., Zhang, W., Liu, B., An, Y., et al. (2022) · Science China Life Sciences · doi:10.1007/s11427-022-2161-3 · Cited by 436 · see citing works
Telomere-related lung fibrosis is diagnostically heterogeneous but uniformly progressive
Newton, C. A., Batra, K., Torrealba, J., Kozlitina, J., Glazer, C. S., Aravena, C., Meyer, K., Raghu, G., Collard, H. R., & Garcia, C. K. (2016) · European Respiratory Journal · doi:10.1183/13993003.00308-2016 · Cited by 400 · see citing works
Sakornsakolpat, P., Prokopenko, D., Lamontagne, M., Reeve, N. F., Guyatt, A. L., Jackson, V. E., Shrine, N. R. G., Qiao, D., Bartz, T. M., Kim, D. K., et al. (2019) · Nature Genetics · doi:10.1038/s41588-018-0342-2 · Cited by 370 · see citing works
Telomeres and age-related disease: how telomere biology informs clinical paradigms
Armanios, M. Y. (2013) · Journal of Clinical Investigation · doi:10.1172/jci66370 · Cited by 346 · see citing works
Self-citationIPF lung fibroblasts have a senescent phenotype
Álvarez, D., Cárdenes, N., Sellarés, J., Bueno, M., Corey, C., Hanumanthu, V. S., Peng, Y., D’Cunha, H., Sembrat, J., Nouraie, M., et al. (2017) · American Journal of Physiology-Lung Cellular and Molecular Physiology · doi:10.1152/ajplung.00220.2017 · Cited by 342 · see citing works
Self-citationTelomere dysfunction causes alveolar stem cell failure
Alder, J. K., Barkauskas, C. E., Limjunyawong, N., Stanley, S. E., Kembou, F., Tuder, R. M., Hogan, B. L. M., Mitzner, W., & Armanios, M. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1504780112 · Cited by 339 · see citing works
Self-citationIdiopathic pulmonary fibrosis: Epithelial-mesenchymal interactions and emerging therapeutic targets
Hewlett, J. C., Kropski, J. A., & Blackwell, T. S. (2018) · Matrix Biology · doi:10.1016/j.matbio.2018.03.021 · Cited by 327 · see citing works
Exploring Animal Models That Resemble Idiopathic Pulmonary Fibrosis
Tashiro, J., Rubio, G. A., LIMPER, A. H., Williams, K. J., Elliot, S. J., Ninou, I., Aidinis, V., Tzouvelekis, A., & Glassberg, M. K. (2017) · Frontiers in Medicine · doi:10.3389/fmed.2017.00118 · Cited by 311 · see citing works
Stuart, B. D., Lee, J. S., Kozlitina, J., Noth, I., Devine, M. S., Glazer, C. S., Torres, F., Kaza, V., Girod, C. E., Jones, K. D., et al. (2014) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(14)70124-9 · Cited by 310 · see citing works
Egea, J., Fabregat, I., Frapart, Y., Ghezzi, P., Görlach, A., Kietzmann, T., Kubaichuk, K. I., Knaus, U. G., López, M. G., González, G. O., et al. (2017) · Redox Biology · doi:10.1016/j.redox.2017.05.007 · Cited by 311 · see citing works
Bone marrow failure and the telomeropathies
Townsley, D. M., Dumitriu, B., & Young, N. S. (2014) · Blood · doi:10.1182/blood-2014-05-526285 · Cited by 290 · see citing works
Abnormal lung aging in chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis
Faner, R., Rojas, M., MacNee, W., & Agustí, A. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201202-0282pp · Cited by 268 · see citing works
Diagnostic utility of telomere length testing in a hospital-based setting
Alder, J. K., Hanumanthu, V. S., Strong, M. A., Dezern, A. E., Stanley, S. E., Takemoto, C. M., Danilova, L., Applegate, C. D., Bolton, S. G., Mohr, D. W., et al. (2018) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1720427115 · Cited by 268 · see citing works
Self-citationDiagnosis of idiopathic pulmonary fibrosis
Choi, H. S. (2021) · Journal of Korean Medical Association · doi:10.5124/jkma.2021.64.4.248 · Cited by 258 · see citing works
Diseases of Pulmonary Surfactant Homeostasis
Whitsett, J. A., Wert, S. E., & Weaver, T. E. (2015) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathol-012513-104644 · Cited by 251 · see citing works
Modeling of fibrotic lung disease using 3D organoids derived from human pluripotent stem cells
Strikoudis, A., Cieślak, A., Loffredo, L., Chen, Y.-W., Patel, N., Saqi, A., Lederer, D. J., & Snoeck, H.-W. (2019) · Cell Reports · doi:10.1016/j.celrep.2019.05.077 · Cited by 242 · see citing works
Mitochondria in the spotlight of aging and idiopathic pulmonary fibrosis
Mora, A. L., Bueno, M., & Rojas, M. M. (2017) · Journal of Clinical Investigation · doi:10.1172/jci87440 · Cited by 234 · see citing works
Juge, P., Borie, R., Kannengiesser, C., Gazal, S., Revy, P., Wémeau-Stervinou, L., Debray, M., Ottaviani, S., Marchand-Adam, S., Nathan, N., et al. (2017) · European Respiratory Journal · doi:10.1183/13993003.02314-2016 · Cited by 234 · see citing works
Cottin, V., Selman, M., Inoue, Y., Wong, A. W., Corte, T. J., Flaherty, K. R., Han, M. K., Jacob, J., Johannson, K. A., Kitaichi, M., et al. (2022) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202206-1041st · Cited by 231 · see citing works
Larkin, E. K., Newman, J. H., Austin, E. D., Hemnes, A. R., Wheeler, L. A., Robbins, I. M., West, J. D., Phillips, J. A., Hamid, R., & Loyd, J. E. (2012) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201205-0886oc · Cited by 219 · see citing works
Stanley, S. E., Gable, D. L., Wagner, C. L., Carlile, T. M., Hanumanthu, V. S., Podlevsky, J. D., Khalil, S. E., Dezern, A. E., Rojas-Durán, M. F., Applegate, C. D., et al. (2016) · Science Translational Medicine · doi:10.1126/scitranslmed.aaf7837 · Cited by 207 · see citing works
Self-citationReview: a meta‐analysis of GWAS and age‐associated diseases
Jeck, W. R., Siebold, A. P., & Sharpless, N. E. (2012) · Aging Cell · doi:10.1111/j.1474-9726.2012.00871.x · Cited by 204 · see citing works
Yanai, H., Shteinberg, A., Porat, Z., Budovsky, A., Braiman, A., Zeische, R., & Fraifeld, V. E. (2015) · Aging · doi:10.18632/aging.100807 · Cited by 198 · see citing works
Luppi, F., Kalluri, M., Faverio, P., Kreuter, M., & Ferrara, G. (2021) · Respiratory Research · doi:10.1186/s12931-021-01711-1 · Cited by 203 · see citing works
Telomere shortening in human diseases
Kong, C. M., Lee, X. W., & Wang, X. (2013) · The FEBS Journal · doi:10.1111/febs.12326 · Cited by 194 · see citing works
The genetic basis of idiopathic pulmonary fibrosis
Kropski, J. A., Blackwell, T. S., & Loyd, J. E. (2015) · European Respiratory Journal · doi:10.1183/09031936.00163814 · Cited by 193 · see citing works
Lung transplantation in telomerase mutation carriers with pulmonary fibrosis
Silhan, L. L., Shah, P. D., Chambers, D. C., Snyder, L. D., Riise, G. C., Wagner, C. L., Hellström‐Lindberg, E., Orens, J. B., Mewton, J. F., Danoff, S. K., et al. (2014) · European Respiratory Journal · doi:10.1183/09031936.00060014 · Cited by 192 · see citing works
Self-citationExome Sequencing Identifies Mutant TINF2 in a Family With Pulmonary Fibrosis
Alder, J. K., Stanley, S. E., Wagner, C. L., Hamilton, M., Hanumanthu, V. S., & Armanios, M. Y. (2014) · CHEST Journal · doi:10.1378/chest.14-1947 · Cited by 181 · see citing works
Self-citationTelomeropathies: An emerging spectrum disorder
Holohan, B., Wright, W. E., & Shay, J. W. (2014) · Journal of Cell Biology · doi:10.1083/jcb.201401012 · Cited by 175 · see citing works
Lung cancer in patients with idiopathic pulmonary fibrosis
Karampitsakos, Τ., Tzilas, V., Tringidou, R., Steiropoulos, P., Aidinis, V., Papiris, S. A., Bouros, D. E., & Τzouvelekis, Α. (2017) · Pulmonary Pharmacology & Therapeutics · doi:10.1016/j.pupt.2017.03.016 · Cited by 170 · see citing works
Mechanisms of telomere loss and their consequences for chromosome instability
Muraki, K., Nyhan, K. C., Han, L., & Murnane, J. P. (2012) · Frontiers in Oncology · doi:10.3389/fonc.2012.00135 · Cited by 162 · see citing works
Telomere-associated aging disorders
Opresko, P. L., & Shay, J. W. (2016) · Ageing Research Reviews · doi:10.1016/j.arr.2016.05.009 · Cited by 155 · see citing works
Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis
Kropski, J. A., Lawson, W. E., Young, L. R., & Blackwell, T. S. (2012) · Disease Models & Mechanisms · doi:10.1242/dmm.010736 · Cited by 154 · see citing works
Telomere structure and telomerase in health and disease
Gomez, D. E., Armando, R. G., Fariña, H. G., Menna, P. L., Cerrudo, C. S., Ghiringhelli, P. D., & Alonso, D. F. (2012) · International Journal of Oncology · doi:10.3892/ijo.2012.1611 · Cited by 145 · see citing works
It all comes together at the ends: Telomerase structure, function, and biogenesis
Podlevsky, J. D., & Chen, J. J. ‐. (2011) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/j.mrfmmm.2011.11.002 · Cited by 144 · see citing works
Biomarkers in idiopathic pulmonary fibrosis
Drakopanagiotakis, F., Wujak, L., Wygrecka, M., & Markart, P. (2018) · Matrix Biology · doi:10.1016/j.matbio.2018.01.023 · Cited by 132 · see citing works
Genetics in Idiopathic Pulmonary Fibrosis Pathogenesis, Prognosis, and Treatment
Kaur, A., Mathai, S. K., & Schwartz, D. A. (2017) · Frontiers in Medicine · doi:10.3389/fmed.2017.00154 · Cited by 130 · see citing works
Telomere-mediated lung disease
Alder, J. K., & Armanios, M. Y. (2022) · Physiological Reviews · doi:10.1152/physrev.00046.2021 · Cited by 126 · see citing works
Self-citationLin, H., & Jiang, S. (2015) · PubMed Central · doi:10.3978/j.issn.2072-1439.2015.04.17 · Cited by 130 · see citing works
Molecular Mechanisms in Progressive Idiopathic Pulmonary Fibrosis
Steele, M. P., & Schwartz, D. A. (2012) · Annual Review of Medicine · doi:10.1146/annurev-med-042711-142004 · Cited by 124 · see citing works
The short and long telomere syndromes: paired paradigms for molecular medicine
Stanley, S. E., & Armanios, M. Y. (2015) · Current Opinion in Genetics & Development · doi:10.1016/j.gde.2015.06.004 · Cited by 123 · see citing works
Self-citationThe Role of Mitochondrial DNA in Mediating Alveolar Epithelial Cell Apoptosis and Pulmonary Fibrosis
Kim, S.-J., Cheresh, P., Jablonski, R. P., Williams, D. B., & Kamp, D. W. (2015) · International Journal of Molecular Sciences · doi:10.3390/ijms160921486 · Cited by 121 · see citing works
Betensley, A. D., Sharif, R., & Karamichos, D. (2016) · Journal of Clinical Medicine · doi:10.3390/jcm6010002 · Cited by 121 · see citing works
Gable, D. L., Gaysinskaya, V. A., Atik, C. C., Talbot, C. C., Kang, B., Stanley, S. E., Pugh, E., Amat-Codina, N., Schenk, K. M., Arcasoy, M. O., et al. (2019) · Genes & Development · doi:10.1101/gad.326785.119 · Cited by 121 · see citing works
Self-citationVeith, C., Boots, A. W., Idris, M., van Schooten, F., & van der Vliet, A. (2019) · Antioxidants and Redox Signaling · doi:10.1089/ars.2019.7742 · Cited by 113 · see citing works
Mechanistic links between aging and lung fibrosis
Thannickal, V. J. (2013) · Biogerontology · doi:10.1007/s10522-013-9451-6 · Cited by 106 · see citing works
MicroRNAs as potential targets for progressive pulmonary fibrosis
Rajasekaran, S., Rajaguru, P., & Paramasivam, S. G. (2015) · Frontiers in Pharmacology · doi:10.3389/fphar.2015.00254 · Cited by 107 · see citing works
Inherited Predisposition to Acute Myeloid Leukemia
Godley, L. A. (2014) · Seminars in Hematology · doi:10.1053/j.seminhematol.2014.08.001 · Cited by 103 · see citing works
Biology of telomeres: lessons from budding yeast
Kupiec, M. (2013) · FEMS Microbiology Reviews · doi:10.1111/1574-6976.12054 · Cited by 100 · see citing works
Smoking and interstitial lung diseases
Margaritopoulos, G. A., Vasarmidi, E., Jacob, J., Wells, A. U., & Antoniou, K. M. (2015) · European Respiratory Review · doi:10.1183/16000617.0050-2015 · Cited by 103 · see citing works
Short Telomere Syndromes in Clinical Practice: Bridging Bench and Bedside
Mangaonkar, A. A., & Patnaik, M. M. (2018) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2018.03.020 · Cited by 99 · see citing works
Fernandez, B. A., Fox, G. A., Bhatia, R., Sala, E., Noble, B., Denic, N., Fernandez, D., Duguid, N., Dohey, A., Kamel, F., et al. (2012) · Respiratory Research · doi:10.1186/1465-9921-13-64 · Cited by 99 · see citing works
Association between telomere length and survival in patients with idiopathic pulmonary fibrosis
Dai, J., Cai, H., Li, H., Zhuang, Y., Min, H., Wen, Y., Yang, J., Gao, Q., Shi, Y. P., & Yi, L. (2015) · Respirology · doi:10.1111/resp.12566 · Cited by 93 · see citing works
Redox mechanisms in age-related lung fibrosis
Kurundkar, A., & Thannickal, V. J. (2016) · Redox Biology · doi:10.1016/j.redox.2016.06.005 · Cited by 88 · see citing works
S2K-Leitlinie zur Diagnostik und Therapie der idiopathischen Lungenfibrose
Behr, J., Günther, A., Ammenwerth, W., Bittmann, I., Bonnet, R. B., Buhl, R., Eickelberg, O., Ewert, R., Gläser, S., Gottlieb, J., et al. (2013) · Pneumologie · doi:10.1055/s-0032-1326009 · Cited by 86 · see citing works
Idiopathic pulmonary fibrosis and lung cancer
Antoniou, K. M., Tomassetti, S., Tsitoura, E., & Vancheri, C. (2015) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000000217 · Cited by 85 · see citing works
Homeostatic nuclear RAGE–ATM interaction is essential for efficient DNA repair
Kumar, V., Fleming, T. H., Terjung, S., Gorzelanny, C., Gebhardt, C., Agrawal, R., Mall, M. A., Ranzinger, J., Zeier, M. G., Madhusudhan, T., et al. (2017) · Nucleic Acids Research · doi:10.1093/nar/gkx705 · Cited by 81 · see citing works
Genetic determinants of pulmonary fibrosis: evolving concepts
Spagnolo, P., Grünewald, J., & du Bois, R. M. (2014) · The Lancet Respiratory Medicine · doi:10.1016/s2213-2600(14)70047-5 · Cited by 81 · see citing works
Pulmonary fibrosis in the era of stratified medicine
Mathai, S. K., Newton, C. A., Schwartz, D. A., & Garcia, C. K. (2016) · Thorax · doi:10.1136/thoraxjnl-2016-209172 · Cited by 77 · see citing works
Genetics of idiopathic pulmonary fibrosis: from mechanistic pathways to personalised medicine
Spagnolo, P., & Cottin, V. (2016) · Journal of Medical Genetics · doi:10.1136/jmedgenet-2016-103973 · Cited by 76 · see citing works
Hung, R. J., Ulrich, C. M., Goode, E. L., Brhane, Y., Muir, K. R., Chan, A. T., Marchand, L. L., Schildkraut, J. M., Witte, J. S., Eeles, R. A., et al. (2015) · JNCI Journal of the National Cancer Institute · doi:10.1093/jnci/djv246 · Cited by 74 · see citing works
Is Progression of Pulmonary Fibrosis due to Ventilation-induced Lung Injury?
Albert, R. K., Smith, B. J., Perlman, C. E., & Schwartz, D. A. (2019) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201903-0497pp · Cited by 72 · see citing works
p16 a biomarker of aging and tolerance for cancer therapy
Muss, H. B., Smitherman, A. B., Wood, W. A., Nyrop, K. A., Tuchman, S. A., Randhawa, P. K., Entwistle, A., Mitin, N., & Shachar, S. S. (2020) · Translational Cancer Research · doi:10.21037/tcr.2020.03.39 · Cited by 68 · see citing works
Pulmonary phenotypes associated with genetic variation in telomere-related genes
Hoffman, T. W., van Moorsel, C. H. M., Borie, R., & Crestani, B. (2018) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000000475 · Cited by 67 · see citing works
Telomere Length Maintenance, Shortening, and Lengthening
Zhao, Z., Pan, X., Liu, L., & Liu, N. (2014) · Journal of Cellular Physiology · doi:10.1002/jcp.24537 · Cited by 66 · see citing works
Understanding interleukin 11 as a disease gene and therapeutic target
Cook, S. A. (2023) · Biochemical Journal · doi:10.1042/bcj20220160 · Cited by 68 · see citing works
Recognizing familial myeloid leukemia in adults
Nickels, E. M., Soodalter, J. A., Churpek, J. E., & Godley, L. A. (2013) · Therapeutic Advances in Hematology · doi:10.1177/2040620713487399 · Cited by 63 · see citing works
Nunès, H., Monnet, I., Kannengiesser, C., Uzunhan, Y., Valeyre, D., Kambouchner, M., & Naccache, J. (2014) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.201309-1724le · Cited by 61 · see citing works
Lin, C., Borensztajn, K., & Spek, C. A. (2017) · Journal of Thrombosis and Haemostasis · doi:10.1111/jth.13623 · Cited by 59 · see citing works
Novel therapeutic strategies for lung disorders associated with airway remodelling and fibrosis
Royce, S. G., Moodley, Y. P., & Samuel, C. S. (2013) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2013.10.008 · Cited by 58 · see citing works
Stanley, S. E., Merck, S. J., & Armanios, M. Y. (2016) · Annals of the American Thoracic Society · doi:10.1513/annalsats.201609-718aw · Cited by 57 · see citing works
Self-citationThe aging lung: tissue telomere shortening in health and disease
Everaerts, S., Lammertyn, E. J., Martens, D. S., Sadeleer, L. D., Maes, K., van Batenburg, A. A., Goldschmeding, R., van Moorsel, C. H. M., Dupont, L., Wuyts, W. A., et al. (2018) · Respiratory Research · doi:10.1186/s12931-018-0794-z · Cited by 55 · see citing works
Telomere and telomerase in chronic liver disease and hepatocarcinoma
Carulli, L. (2014) · World Journal of Gastroenterology · doi:10.3748/wjg.v20.i20.6287 · Cited by 54 · see citing works
Pirfenidone: significant treatment effects in idiopathic pulmonary fibrosis
Hilberg, O., Simonsen, U., du Bois, R. M., & Bendstrup, E. M. (2012) · The Clinical Respiratory Journal · doi:10.1111/j.1752-699x.2012.00302.x · Cited by 54 · see citing works
Predictive factors and prognostic effect of telomere shortening in pulmonary fibrosis
Planas‐Cerezales, L., Arias‐Salgado, E. G., Buendía-Roldán, I., Montes‐Worboys, A., Esquinas, C., Vicens‐Zygmunt, V., Hernáiz, P. L., Sanuy, R. L., Leiro‐Fernández, V., Balcells, E., et al. (2018) · Respirology · doi:10.1111/resp.13423 · Cited by 54 · see citing works
Genetic Interstitial Lung Disease
Devine, M., & Garcia, C. K. (2011) · Clinics in Chest Medicine · doi:10.1016/j.ccm.2011.11.001 · Cited by 53 · see citing works
A role for telomere length and chromosomal damage in idiopathic pulmonary fibrosis
McDonough, J. E., Martens, D. S., Tanabe, N., Ahangari, F., Verleden, S. E., Maes, K., Verleden, G. M., Kaminski, N., Hogg, J. C., Nawrot, T. S., et al. (2018) · Respiratory Research · doi:10.1186/s12931-018-0838-4 · Cited by 49 · see citing works
Evaluation and Management of Hematopoietic Failure in Dyskeratosis Congenita
Agarwal, S. (2018) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2018.04.003 · Cited by 49 · see citing works
Tomos, I. P., Dimakopoulou, K., Manali, E. D., Papiris, S. A., & Karakatsani, A. (2021) · Environmental Health · doi:10.1186/s12940-021-00786-z · Cited by 48 · see citing works
Hepatic manifestations of telomere biology disorders
Patnaik, M. M., Kamath, P. S., & Simonetto, D. A. (2018) · Journal of Hepatology · doi:10.1016/j.jhep.2018.05.006 · Cited by 44 · see citing works
Arish, N., Petukhov, D., & Wallach‐Dayan, S. B. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20122996 · Cited by 45 · see citing works
KLF4 regulates TERT expression in alveolar epithelial cells in pulmonary fibrosis
Wang, H., Xu, H., Lyu, W., Xu, Q., Fan, S., Chen, H., Wang, D., Chen, J., & Dai, J. (2022) · Cell Death and Disease · doi:10.1038/s41419-022-04886-7 · Cited by 46 · see citing works
Gaysinskaya, V. A., Stanley, S. E., Adam, S. S., & Armanios, M. Y. (2020) · CHEST Journal · doi:10.1016/j.chest.2020.07.025 · Cited by 43 · see citing works
Self-citationTeichroeb, J. H., Kim, J., & Betts, D. H. (2016) · RNA Biology · doi:10.1080/15476286.2015.1134413 · Cited by 43 · see citing works
Dai, J., Cai, H., Zhuang, Y., Wu, Y., Min, H., Li, J., Shi, Y. P., Gao, Q., & Yi, L. (2014) · Respirology · doi:10.1111/resp.12422 · Cited by 41 · see citing works
Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications
Spagnolo, P., Rossi, G., & Cavazza, A. (2014) · Expert Review of Clinical Immunology · doi:10.1586/1744666x.2014.917050 · Cited by 42 · see citing works
Telomere dysfunction implicates POT1 in patients with idiopathic pulmonary fibrosis
Kelich, J. M., Aramburu, T. M., van der Vis, J. J., Showe, L. C., Kossenkov, A. V., van der Smagt, J. J., Massink, M. P. G., Schoemaker, A. E., Hennekam, E. A. M., Veltkamp, M., et al. (2022) · The Journal of Experimental Medicine · doi:10.1084/jem.20211681 · Cited by 42 · see citing works
The wide-ranging clinical implications of the Short Telomere Syndromes
Barbaro, P. M., Ziegler, D. S., & Reddel, R. R. (2016) · Internal Medicine Journal · doi:10.1111/imj.12868 · Cited by 40 · see citing works
The Possible Pathogenesis of Idiopathic Pulmonary Fibrosis consideringMUC5B
Zhang, Q., Wang, Y., Qu, D., Yu, J., & Yang, J. (2019) · BioMed Research International · doi:10.1155/2019/9712464 · Cited by 40 · see citing works
Genetic susceptibility and pulmonary fibrosis
Mathai, S. K., Schwartz, D. A., & Warg, L. A. (2014) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000000074 · Cited by 39 · see citing works
Tao, N., Li, K., Liu, J., Fan, G., & Sun, T. (2021) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2021.02.127 · Cited by 39 · see citing works
Trade-offs in aging lung diseases
van Moorsel, C. H. M. (2018) · Current Opinion in Pulmonary Medicine · doi:10.1097/mcp.0000000000000476 · Cited by 39 · see citing works
Hereditary Predisposition to Hematopoietic Neoplasms
Mangaonkar, A. A., & Patnaik, M. M. (2020) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2019.12.013 · Cited by 37 · see citing works
- Authors
- Armanios, M.
- Affiliations
- Johns Hopkins University (US)
- Corresponding author
- Armanios, Mary
- ORCID
- Armanios, Mary
- Venue
- Mutation Research - Fundamental and Molecular Mechanisms of Mutagenesis
- Publisher
- Elsevier BV
- Published
- 2012-02
- Volume
- 730
- Issue
- 1-2
- Pages
- 52-58
- ISSN
- 0027-5107 (ISSN-L)
- 1386-1964
- PMC
- PMC3292861
- OpenAlex
- W1973305610
- Cited by
- 245
- References
- 56
- Citations by year
- 2026: 1 · 2025: 11 · 2024: 4 · 2023: 13 · 2022: 19 · 2021: 19 · 2020: 16 · 2019: 25 · 2018: 18 · 2017: 18 · 2016: 18 · 2015: 25 · 2014: 24 · 2013: 16 · 2012: 16
- FWCI
- 1.99
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Mutation research. Fundamental and molecular mechanisms of mutagenesis (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Maryland Stem Cell Research Fund
- Flight Attendant Medical Research Institute
- Topics
- Health Sciences › Medicine › Physiology › Telomeres, Telomerase, and Senescence
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Occupational and environmental lung diseases
- Keywords
- idiopathic pulmonary fibrosis, telomerase, hTERT, hTR, germline mutations, telomere shortening, bone marrow failure, cryptogenic cirrhosis, genetic anticipation
- MeSH terms
- Age Factors, Bone Marrow Diseases, Emphysema, Genes, Dominant, Humans, Pulmonary Fibrosis, Telomerase, Dyskeratosis Congenita, Genetic Predisposition to Disease, Telomere Shortening
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