Zobrazeno 1 - 10
of 23
pro vyhledávání: '"Pamela Hale"'
Autor:
Yan Wang, Murat C Cobanoglu, Jie Li, Tunda Hidvegi, Pamela Hale, Michael Ewing, Andrew S Chu, Zhenwei Gong, Radhika Muzumdar, Stephen C Pak, Gary A Silverman, Ivet Bahar, David H Perlmutter
Publikováno v:
PLoS ONE, Vol 14, Iss 1, p e0209748 (2019)
The classical form of α1-antitrypsin deficiency (ATD) is characterized by intracellular accumulation of the misfolded variant α1-antitrypsin Z (ATZ) and severe liver disease in some of the affected individuals. In this study, we investigated the po
Externí odkaz:
https://doaj.org/article/15ce59d7c3b44813913242c9f529345a
Autor:
Jie Li, Stephen C Pak, Linda P O'Reilly, Joshua A Benson, Yan Wang, Tunda Hidvegi, Pamela Hale, Christine Dippold, Michael Ewing, Gary A Silverman, David H Perlmutter
Publikováno v:
PLoS ONE, Vol 9, Iss 1, p e87260 (2014)
The classical form of α1-antitrypsin deficiency (ATD) is associated with hepatic fibrosis and hepatocellular carcinoma. It is caused by the proteotoxic effect of a mutant secretory protein that aberrantly accumulates in the endoplasmic reticulum of
Externí odkaz:
https://doaj.org/article/c378e72431a64ece8d6c33c6f47ce71c
Autor:
David A. Rudnick, Jiansheng Huang, Tunda Hidvegi, Andrew S. Chu, Pamela Hale, Admire Munanairi, Dennis J. Dietzen, Paul F. Cliften, Eric Tycksen, Andrew J. Lutkewitte, Brian N. Finck, Stephen C. Pak, Gary A. Silverman, David H. Perlmutter
Publikováno v:
Gastroenterology. 163(1)
Insulin signaling is known to regulate essential proteostasis mechanisms.The analyses here examined effects of insulin signaling in the PiZ mouse model of α1-antitrypsin deficiency in which hepatocellular accumulation and proteotoxicity of the misfo
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fa4b6cb469d84421c34ab22fe9ce0a95
https://pubmed.ncbi.nlm.nih.gov/35500618
https://pubmed.ncbi.nlm.nih.gov/35500618
Autor:
Pamela Hale, Cliff J. Luke, Murat Can Cobanoglu, Mark T. Miedel, Gary A. Silverman, Olivia S. Long, Joshua A. Benson, Ivet Bahar, Linda P. O’Reilly, David H. Perlmutter, Stephen C. Pak
Publikováno v:
Human Molecular Genetics. 23:5123-5132
α1-Antitrypsin deficiency (ATD) is a common genetic disorder that can lead to end-stage liver and lung disease. Although liver transplantation remains the only therapy currently available, manipulation of the proteostasis network (PN) by small molec
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c63fcbdba974e33bfbaa4f2280855025
https://doi.org/10.1093/hmg/ddu236
https://doi.org/10.1093/hmg/ddu236
An analog of glibenclamide selectively enhances autophagic degradation of misfolded α1-antitrypsin Z
Autor:
Tunda Hidvegi, Ivet Bahar, Stephen C. Pak, Jie Li, David H. Perlmutter, Pamela Hale, Murat Can Cobanoglu, Andrew S. Chu, Radhika Muzumdar, Gary A. Silverman, Yan Wang, Michael Ewing, Zhenwei Gong
Publikováno v:
PLoS ONE
PLoS ONE, Vol 14, Iss 1, p e0209748 (2019)
PLoS ONE, Vol 14, Iss 1, p e0209748 (2019)
The classical form of α1-antitrypsin deficiency (ATD) is characterized by intracellular accumulation of the misfolded variant α1-antitrypsin Z (ATZ) and severe liver disease in some of the affected individuals. In this study, we investigated the po
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::32ee8e5dd7dd1df0f732af39a74aafdf
https://doi.org/10.1371/journal.pone.0209748
https://doi.org/10.1371/journal.pone.0209748
Autor:
Michael Ewing, Caroline Beckett, Christine S. Dippold, Christina Goldbach, Simon C. Watkins, Carolyn Kemp, Nicholas Maurice, Tunda Hidvegi, Amitava Mukherjee, George K. Michalopoulos, Pamela Hale, David H. Perlmutter
Publikováno v:
Science. 329:229-232
Correcting a Liver Problem The classical form of α 1 -antitrypsin (AT) deficiency is caused by a point mutation that alters the folding and causes intracellular aggregation of AT—an abundant liver-derived plasma glycoprotein. AT deficiency is the
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::97720ded03580b55a6bdf95a82f0a277
https://doi.org/10.1126/science.1190354
https://doi.org/10.1126/science.1190354
Autor:
Pamela Hale, Peter M. George, Mohamed Saleem, John G. Lewis, Guy P Mulligan, Christopher M. Florkowski
Publikováno v:
Annals of Clinical Biochemistry: International Journal of Laboratory Medicine. 46:172-175
Interference in immunoassays is a widely recognized problem, which could potentially lead to unnecessary investigations and treatment. We describe a case where interference in a cortisol immunoassay led to a falsely low serum cortisol concentration a
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a030f2bbf3b5ad616c92c804843098c2
https://doi.org/10.1258/acb.2008.008224
https://doi.org/10.1258/acb.2008.008224
Autor:
Pamela Hale, Michael Ewing, Caroline Beckett, David H. Perlmutter, Tunda Hidvegi, Karoly Mirnics
Publikováno v:
Journal of Biological Chemistry. 282:27769-27780
In the classical form of alpha(1)-antitrypsin deficiency, a mutant protein accumulates in a polymerized form in the endoplasmic reticulum (ER) of liver cells causing liver damage and carcinogenesis by a gain-of-toxic function mechanism. Recent studie
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::eaddecdbb89148454439b017c18575a4
https://doi.org/10.1074/jbc.m704330200
https://doi.org/10.1074/jbc.m704330200
Autor:
Darrell N. Kotton, Alejandro Soto-Gutierrez, Carol Feghali-Bostwick, Wanquan Wu, Pamela Hale, Jayanta Roy-Chowdhury, Ira J. Fox, Andrew A. Wilson, Bing Han, David H. Perlmutter, Souvik Chakraborty, Tafaleng Edgar Naoe, Stephen C. Strom, Masaki Nagaya, Donna B. Stolz
In the classical form of α1-antitrypsin deficiency (ATD), aberrant intracellular accumulation of misfolded mutant α1-antitrypsin Z (ATZ) in hepatocytes causes hepatic damage by a gain-of-function, “proteotoxic” mechanism. While some ATD patient
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d904f6896fc66a44534d5cfac9abde3a
https://europepmc.org/articles/PMC4482790/
https://europepmc.org/articles/PMC4482790/
Autor:
Samuel A. Yousem, Nunzia Pastore, Pamela Hale, Steven D. Shapiro, Patrizia Annunziata, Michael Ewing, Nicola Brunetti-Pierri, Stephen C. Pak, Naftali Kaminski, Donna B. Stolz, Jieru Wang, Joseph M. Pilewski, Adriana S. Leme, Evelyn Akpadock Garchar, A. McGarry Houghton, David H. Perlmutter, Gary A. Silverman, Tunda Hidvegi, Houming Cai, John F. Alcorn
Recent studies have shown that autophagy mitigates the pathological effects of proteinopathies in the liver, heart, and skeletal muscle but this has not been investigated for proteinopathies that affect the lung. This may be due at least in part to t
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d6a3f2445bf8ce202154e7ce4dababe9
http://hdl.handle.net/11588/614053
http://hdl.handle.net/11588/614053