Zobrazeno 1 - 9
of 9
pro vyhledávání: '"Olivia S. Long"'
Autor:
Mark T Miedel, Nathan J Graf, Kate E Stephen, Olivia S Long, Stephen C Pak, David H Perlmutter, Gary A Silverman, Cliff J Luke
Publikováno v:
PLoS ONE, Vol 7, Iss 7, p e40145 (2012)
Endoplasmic-reticulum associated degradation (ERAD) is a major cellular misfolded protein disposal pathway that is well conserved from yeast to mammals. In yeast, a mutant of carboxypeptidase Y (CPY*) was found to be a luminal ER substrate and has se
Externí odkaz:
https://doaj.org/article/b277c57644ed4e9abc486f94a5552bea
Autor:
Sager J Gosai, Joon Hyeok Kwak, Cliff J Luke, Olivia S Long, Dale E King, Kevin J Kovatch, Paul A Johnston, Tong Ying Shun, John S Lazo, David H Perlmutter, Gary A Silverman, Stephen C Pak
Publikováno v:
PLoS ONE, Vol 5, Iss 11, p e15460 (2010)
The development of preclinical models amenable to live animal bioactive compound screening is an attractive approach to discovering effective pharmacological therapies for disorders caused by misfolded and aggregation-prone proteins. In general, howe
Externí odkaz:
https://doaj.org/article/84a23b8725da453282e732e846ecd8d0
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
Autor:
David H. Perlmutter, Cliff J. Luke, Olivia S. Long, Anne C. Vetica, Sangeeta R. Bhatia, Gary A. Silverman, Stephen C. Pak
Publikováno v:
Pediatric Research. 65:10-18
As an experimental system, Caenorhabditis elegans, offers a unique opportunity to interrogate in vivo the genetic and molecular functions of human disease-related genes. For example, C. elegans has provided crucial insights into fundamental biologica
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::30128bab4f9ae857abd30e70952c3287
https://doi.org/10.1203/pdr.0b013e31819009b0
https://doi.org/10.1203/pdr.0b013e31819009b0
Autor:
Stephen C. Pak, Robert Barstead, Gary Moulder, Terra L. Naviglia, Yuko S. Askew, Olivia S. Long, Anne C. Vetica, Shila M. Nobar, David J. Askew, Simon C. Watkins, Donna B. Stolz, Gary A. Silverman, Cliff J. Luke, Dieter Brömme
Publikováno v:
Cell. 130(6):1108-1119
Summary Extracellular serpins such as antithrombin and α1-antitrypsin are the quintessential regulators of proteolytic pathways. In contrast, the biological functions of the intracellular serpins remain obscure. We now report that the C. elegans int
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3fbe7b2fd7b21ed3053156bf05318ba6
Autor:
Yan Wang, Mark T. Miedel, Gary A. Silverman, David H. Perlmutter, Simon C. Watkins, Linda P. O’Reilly, Joshua A. Benson, Stephen C. Pak, Stephan Züchner, Jie Li, Joon Hyeok Kwak, Sager J. Gosai, Olivia S. Long, Anne C. Vetica, Donna B. Stolz, Cliff J. Luke
Publikováno v:
Human molecular genetics. 23(19)
The accumulation of serpin oligomers and polymers within the endoplasmic reticulum (ER) causes cellular injury in patients with the classical form α1-antitrypsin deficiency (ATD). To better understand the cellular and molecular genetic aspects of th
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b5f7244781cfea148dded5410106f88e
https://pubmed.ncbi.nlm.nih.gov/24838286
https://pubmed.ncbi.nlm.nih.gov/24838286
Autor:
David H. Perlmutter, Joon Hyeok Kwak, Stephen C. Pak, Olivia S. Long, Gary A. Silverman, Dale E. King, Sager J. Gosai
Publikováno v:
Methods in enzymology. 499
Protein misfolding, polymerization, and/or aggregation are hallmarks of serpinopathies and many other human genetic disorders including Alzheimer's, Huntington's, and Parkinson's disease. While higher organism models have helped shape our understandi
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::006e459eb5ef97e19a6430e120855c1b
https://pubmed.ncbi.nlm.nih.gov/21683258
https://pubmed.ncbi.nlm.nih.gov/21683258
Autor:
Tong Ying Shun, David H. Perlmutter, Cliff J. Luke, Paul A. Johnston, Kevin J. Kovatch, Gary A. Silverman, Dale E. King, Olivia S. Long, Joon Hyeok Kwak, John S. Lazo, Sager J. Gosai, Stephen C. Pak
Publikováno v:
PLoS ONE, Vol 5, Iss 11, p e15460 (2010)
PLoS ONE
PLoS ONE
The development of preclinical models amenable to live animal bioactive compound screening is an attractive approach to discovering effective pharmacological therapies for disorders caused by misfolded and aggregation-prone proteins. In general, howe
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3fe4268ce9790f49941985e5bcbc9947
http://europepmc.org/articles/PMC2980495?pdf=render
http://europepmc.org/articles/PMC2980495?pdf=render
Autor:
Nathan J. Graf, Stephen C. Pak, Mark T. Miedel, Olivia S. Long, Gary A. Silverman, Cliff J. Luke, David H. Perlmutter, Kate E. Stephen
Publikováno v:
PLoS ONE, Vol 7, Iss 7, p e40145 (2012)
PLoS ONE
PLoS ONE
Endoplasmic-reticulum associated degradation (ERAD) is a major cellular misfolded protein disposal pathway that is well conserved from yeast to mammals. In yeast, a mutant of carboxypeptidase Y (CPY*) was found to be a luminal ER substrate and has se
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::138dd891d8bb22ddc914c3765aae195c
https://doi.org/10.1371/journal.pone.0040145
https://doi.org/10.1371/journal.pone.0040145