Zobrazeno 1 - 10
of 146
pro vyhledávání: '"Stephen P. Bottomley"'
Autor:
Andre L. Samson, Bosco Ho, Amanda E. Au, Simone M. Schoenwaelder, Mark J. Smyth, Stephen P. Bottomley, Oded Kleifeld, Robert L. Medcalf
Publikováno v:
Open Biology, Vol 6, Iss 11 (2016)
Amyloidogenic protein aggregation impairs cell function and is a hallmark of many chronic degenerative disorders. Protein aggregation is also a major event during acute injury; however, unlike amyloidogenesis, the process of injury-induced protein ag
Externí odkaz:
https://doaj.org/article/37f23dd65fe14990a71f4f507aa866be
Autor:
Andre L. Samson, Anja S. Knaupp, Maithili Sashindranath, Rachael J. Borg, Amanda E.-L. Au, Elisa J. Cops, Helen M. Saunders, Stephen H. Cody, Catriona A. McLean, Cameron J. Nowell, Victoria A. Hughes, Stephen P. Bottomley, Robert L. Medcalf
Publikováno v:
Cell Reports, Vol 2, Iss 4, Pp 889-901 (2012)
Cellular injury causes a myriad of processes that affect proteostasis. We describe nucleocytoplasmic coagulation (NCC), an intracellular disulfide-dependent protein crosslinking event occurring upon late-stage cell death that orchestrates the proteol
Externí odkaz:
https://doaj.org/article/c012729ca9db4e86aa500df9790bce9b
Publikováno v:
PLoS ONE, Vol 9, Iss 9, p e102617 (2014)
α1-Antitrypsin (α1AT) deficiency, the most common serpinopathy, results in both emphysema and liver disease. Over 90% of all clinical cases of α1AT deficiency are caused by the Z variant in which Glu342, located at the top of s5A, is replaced by a
Externí odkaz:
https://doaj.org/article/1441956e44c84a3689847f3755838e6e
Publikováno v:
PLoS ONE, Vol 8, Iss 1, p e54766 (2013)
α(1)-Antitrypsin, the archetypal member of the serpin superfamily, is a metastable protein prone to polymerization when exposed to stressors such as elevated temperature, low denaturant concentrations or through the presence of deleterious mutations
Externí odkaz:
https://doaj.org/article/32cd3d06b9dd40a6a3c701ad211ce205
Autor:
Martin J. Scanlon, Stephen J. Headey, Stephen P. Bottomley, Amy L. Robertson, Robert N. Pike, Lakshmi C. Wijeyewickrema, Natasha M. Ng
Publikováno v:
Biochimie. 122:227-234
Proteolysis has a critical role in transmitting information within a biological system and therefore an important element of biology is to determine the subset of proteins amenable to proteolysis. Until recently, it has been thought that proteases cl
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::042be4f00d636de725929a7416228594
https://doi.org/10.1016/j.biochi.2015.09.021
https://doi.org/10.1016/j.biochi.2015.09.021
Autor:
Oded Kleifeld, Mark J. Smyth, Amanda E. Au, Andre L. Samson, Bosco K. Ho, Stephen P. Bottomley, Robert L. Medcalf, Simone M. Schoenwaelder
Publikováno v:
Open Biology
Open Biology, Vol 6, Iss 11 (2016)
Open Biology, Vol 6, Iss 11 (2016)
Amyloidogenic protein aggregation impairs cell function and is a hallmark of many chronic degenerative disorders. Protein aggregation is also a major event during acute injury; however, unlike amyloidogenesis, the process of injury-induced protein ag
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dcf713d25a90f916bad8c0e272214ecb
https://zenodo.org/record/1065526
https://zenodo.org/record/1065526
Autor:
Anja S Knaupp, Stephen P. Bottomley
Publikováno v:
Journal of Molecular Biology. 413:888-898
The presence of the Z mutation (Glu342Lys) is responsible for more than 95% of α(1)-antitrypsin (α(1)AT) deficiency cases. It leads to increased polymerization of the serpin α(1)AT during its synthesis and in circulation. It has been proposed that
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a4c91d5020d88bbb7444052e8f302f8d
https://doi.org/10.1016/j.jmb.2011.09.013
https://doi.org/10.1016/j.jmb.2011.09.013
Publikováno v:
Journal of Molecular Biology. 413:879-887
The nine polyglutamine (polyQ) neurodegenerative diseases are caused in part by a gain-of-function mechanism involving protein misfolding, the deposition of β-sheet-rich aggregates and neuronal toxicity. While previous experimental evidence suggests
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::71e2c8269730f36af21e758840adfc6f
https://doi.org/10.1016/j.jmb.2011.09.014
https://doi.org/10.1016/j.jmb.2011.09.014
Autor:
Susanne C. Feil, Guido Hansen, Glenn A. Powers, Michael W. Parker, Mary Catherine Pearce, Stephen P. Bottomley
Publikováno v:
Journal of Molecular Biology. 403:459-467
The native serpin state is kinetically trapped. However, under mildly destabilizing conditions, the conformational landscape changes, and a number of nonnative conformations with increased stability can be readily formed. The ability to undergo struc
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a4306d652810e86f5a9e9408dfcb6f6e
https://doi.org/10.1016/j.jmb.2010.09.007
https://doi.org/10.1016/j.jmb.2010.09.007
Autor:
Amy L. Robertson, Stephen P. Bottomley
Publikováno v:
Current Medicinal Chemistry. 17:3058-3068
Protein aggregation is a key mechanism involved in neurodegeneration associated with Alzheimer's, Parkinson's and Huntington's diseases. Nine diseases (including Huntington's) arise from polyglutamine (polyQ) expansion above a repeat threshold of app
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::36fcafbc3cdddb2bfb53d288e5b2c4fd
https://doi.org/10.2174/092986710791959800
https://doi.org/10.2174/092986710791959800