Zobrazeno 1 - 10
of 38
pro vyhledávání: '"Hilda Brown"'
Autor:
Anthony M. Crown, Brittany L. Roberts, Keith Crosby, Hilda Brown, Jacob I. Ayers, P. John Hart, David R. Borchelt
Publikováno v:
G3: Genes, Genomes, Genetics, Vol 9, Iss 3, Pp 719-728 (2019)
Mutations in more than 80 different positions in superoxide dismutase 1 (SOD1) have been associated with amyotrophic lateral sclerosis (fALS). There is substantial evidence that a common consequence of these mutations is to induce the protein to misf
Externí odkaz:
https://doaj.org/article/1f7133afa82c457cb6d8df9f41d1ca3a
Autor:
Zachary Osking, Jacob I. Ayers, Ryan Hildebrandt, Kristen Skruber, Hilda Brown, Daniel Ryu, Amanda R. Eukovich, Todd E. Golde, David R. Borchelt, Tracy-Ann Read, Eric A. Vitriol
Publikováno v:
iScience, Vol 11, Iss , Pp 294-304 (2019)
Summary: Amyotrophic lateral sclerosis (ALS) is a progressive, fatal neurodegenerative disease characterized by motor neuron cell death. However, not all motor neurons are equally susceptible. Most of what we know about the surviving motor neurons co
Externí odkaz:
https://doaj.org/article/d70657e976174f1195ce5d2223963fe0
Autor:
Christina Moloney, Sruti Rayaprolu, John Howard, Susan Fromholt, Hilda Brown, Matt Collins, Mariela Cabrera, Colin Duffy, Zoe Siemienski, Dave Miller, David R. Borchelt, Jada Lewis
Publikováno v:
Acta Neuropathologica Communications, Vol 6, Iss 1, Pp 1-13 (2018)
Abstract Mutations in MATR3 have been associated with amyotrophic lateral sclerosis (ALS) as well as a form of distal myopathy termed vocal cord pharyngeal distal myopathy (VCPDM). To begin to understand how mutations in MATR3 may cause disease, here
Externí odkaz:
https://doaj.org/article/b6928d9d6c3347e99487cc6b79af70de
Autor:
Anthony Crown, Luke McAlary, Eric Fagerli, Hilda Brown, Justin J Yerbury, Ahmad Galaleldeen, Neil R Cashman, David R Borchelt, Jacob I Ayers
Publikováno v:
PLoS ONE, Vol 15, Iss 1, p e0227655 (2020)
Mutations in Cu/Zn superoxide dismutase 1 (SOD1) associated with familial amyotrophic lateral sclerosis cause the protein to aggregate via a prion-like process in which soluble molecules are recruited to aggregates by conformational templating. These
Externí odkaz:
https://doaj.org/article/2780c523d04745fcb6fa967d68601d3b
Autor:
Keith Crosby, Anthony M Crown, Brittany L Roberts, Hilda Brown, Jacob I Ayers, David R Borchelt
Publikováno v:
PLoS ONE, Vol 13, Iss 11, p e0206751 (2018)
Mutations in superoxide dismutase 1 (SOD1) associated with familial amyotrophic lateral sclerosis (fALS) induce the protein to misfold and aggregate. Missense mutations at more than 80 different amino acid positions have been associated with disease.
Externí odkaz:
https://doaj.org/article/414dc445bd0343c5bbcff34534cf4580
Autor:
Christina Moloney, Sruti Rayaprolu, John Howard, Susan Fromholt, Hilda Brown, Matt Collins, Mariela Cabrera, Colin Duffy, Zoe Siemienski, Dave Miller, Maurice S. Swanson, Lucia Notterpek, David R. Borchelt, Jada Lewis
Publikováno v:
Acta Neuropathologica Communications, Vol 5, Iss 1, Pp 1-1 (2017)
Abstract The authors are retracting this article. The article describes mice expressing wild-type human MATR3. However, since publication the authors have become aware that all of the lines of mice described express human MATR3 containing the F115C m
Externí odkaz:
https://doaj.org/article/d64237b7552645d7a5d2dfd84b751f97
Publikováno v:
PLoS ONE, Vol 8, Iss 12, p e83981 (2013)
By mechanisms yet to be discerned, the co-expression of high levels of wild-type human superoxide dismutase 1 (hSOD1) with variants of hSOD1 encoding mutations linked familial amyotrophic lateral sclerosis (fALS) hastens the onset of motor neuron deg
Externí odkaz:
https://doaj.org/article/dbc65720c8854e68a4a099fe2df2b103
Autor:
Guilian Xu, Stanley M. Stevens, Firas Kobiessy, Hilda Brown, Scott McClung, Mark S. Gold, David R. Borchelt
Publikováno v:
PLoS ONE, Vol 7, Iss 11 (2012)
Externí odkaz:
https://doaj.org/article/c9726c5f39f84523859c477ccaa14e07
Identification of proteins sensitive to thermal stress in human neuroblastoma and glioma cell lines.
Autor:
Guilian Xu, Stanley M Stevens, Firas Kobeissy, Hilda Brown, Scott McClung, Mark S Gold, David R Borchelt
Publikováno v:
PLoS ONE, Vol 7, Iss 11, p e49021 (2012)
Heat-shock is an acute insult to the mammalian proteome. The sudden elevation in temperature has far-reaching effects on protein metabolism, leads to a rapid inhibition of most protein synthesis, and the induction of protein chaperones. Using heat-sh
Externí odkaz:
https://doaj.org/article/494ece5b40d9465786fdb041dcdd3909
Autor:
M. Carolina Gallego-Iradi, Edgardo Rodriguez-Lebron, Haley Strunk, Anthony M. Crown, Rachel Davila, David R. Borchelt, Hilda Brown
Publikováno v:
Laboratory investigation; a journal of technical methods and pathology
RNA binding proteins associated with amyotrophic lateral sclerosis (ALS) and muscle myopathy possess sequence elements that are low in complexity, or bear resemblance to yeast prion domains. These sequence elements appear to mediate phase separation