Zobrazeno 1 - 10
of 305
pro vyhledávání: '"Henry L Paulson"'
Autor:
Easton Wollney, Kaitlin Sovich, Brian LaBarre, Susan M Maixner, Henry L Paulson, Carol Manning, Julie A Fields, Angela Lunde, Bradley F Boeve, James E Galvin, Angela S Taylor, Zhigang Li, Hannah J Fechtel, Melissa J Armstrong
Publikováno v:
PLoS ONE, Vol 19, Iss 8, p e0309530 (2024)
BackgroundDementia with Lewy bodies (DLB) is one of the most common degenerative dementias, but research on end-of-life experiences for people with DLB and their caregivers is limited.MethodDyads of individuals with moderate-advanced DLB and their pr
Externí odkaz:
https://doaj.org/article/47dd0a3e12d14e359085115e15990800
Autor:
Zhigang Li, Melissa J Armstrong, Henry L Paulson, Susan M Maixner, Julie A Fields, Angela M Lunde, Bradley F Boeve, Carol Manning, James E Galvin, Angela S Taylor
Publikováno v:
BMJ Open, Vol 11, Iss 5 (2021)
Introduction Dementia with Lewy bodies (DLB) is one of the most common degenerative dementias. Despite the fact that most individuals with DLB die from complications of the disease, little is known regarding what factors predict impending end of life
Externí odkaz:
https://doaj.org/article/59efe3e85a4044bdafd588a26bd084e2
Publikováno v:
PLoS ONE, Vol 13, Iss 9, p e0204438 (2018)
Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disorder caused by a polyglutamine-encoding CAG repeat expansion in the ATXN3 gene which encodes the deubiquitinating enzyme, ATXN3. Several mechanisms have been propose
Externí odkaz:
https://doaj.org/article/dbb46c065ea745759fab0c7153328a07
Autor:
Brittany N Flores, Mark E Dulchavsky, Amy Krans, Michael R Sawaya, Henry L Paulson, Peter K Todd, Sami J Barmada, Magdalena I Ivanova
Publikováno v:
PLoS ONE, Vol 11, Iss 10, p e0165084 (2016)
Hexanucleotide repeat expansions in C9orf72 are the most common inherited cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The expansions elicit toxicity in part through repeat-associated non-AUG (RAN) translation of th
Externí odkaz:
https://doaj.org/article/fe73098c17dd4b6d8f8a53920cde6af8
Autor:
Takahiro Seki, Ken-ich Yoshino, Shigeru Tanaka, Eisuke Dohi, Tomoya Onji, Kazuhiro Yamamoto, Izumi Hide, Henry L Paulson, Naoaki Saito, Norio Sakai
Publikováno v:
PLoS ONE, Vol 7, Iss 2, p e31232 (2012)
BACKGROUND: Chaperone-mediated autophagy (CMA) is a selective autophagy-lysosome protein degradation pathway. The role of CMA in normal neuronal functions and in neural disease pathogenesis remains unclear, in part because there is no available metho
Externí odkaz:
https://doaj.org/article/998a4e3d7def49dcbb78b9980c8eceb4
Publikováno v:
PLoS ONE, Vol 7, Iss 9, p e43563 (2012)
Alterations in the ubiquitin-proteasome system (UPS) have been reported in several neurodegenerative disorders characterized by protein misfolding and aggregation, including the polylgutamine diseases. Machado-Joseph disease (MJD) or Spinocerebellar
Externí odkaz:
https://doaj.org/article/953a45f3f77a47878c368799e837f737
Autor:
Peter K Todd, Seok Yoon Oh, Amy Krans, Udai B Pandey, Nicholas A Di Prospero, Kyung-Tai Min, J Paul Taylor, Henry L Paulson
Publikováno v:
PLoS Genetics, Vol 6, Iss 12, p e1001240 (2010)
Fragile X Tremor Ataxia Syndrome (FXTAS) is a common inherited neurodegenerative disorder caused by expansion of a CGG trinucleotide repeat in the 5'UTR of the fragile X syndrome (FXS) gene, FMR1. The expanded CGG repeat is thought to induce toxicity
Externí odkaz:
https://doaj.org/article/40811b280a13446ba4330e81a3fb5fa6
Publikováno v:
PLoS ONE, Vol 5, Iss 10, p e13695 (2010)
Protein context clearly influences neurotoxicity in polyglutamine diseases, but the contribution of alternative splicing to this phenomenon has rarely been investigated. Ataxin-3, a deubiquitinating enzyme and the disease protein in SCA3, is alternat
Externí odkaz:
https://doaj.org/article/358dde08152e498c9a9654a8263d97ad
Autor:
Giuseppe Nicastro, Sokol V Todi, Ezgi Karaca, Alexandre M J J Bonvin, Henry L Paulson, Annalisa Pastore
Publikováno v:
PLoS ONE, Vol 5, Iss 8, p e12430 (2010)
Ataxin-3, the disease protein in the neurodegenerative disorder Spinocerebellar Ataxia Type 3 or Machado Joseph disease, is a cysteine protease implicated in the ubiquitin proteasome pathway. It contains multiple ubiquitin binding sites through which
Externí odkaz:
https://doaj.org/article/3daa2ae6eaab4d869ee72031e12f8395
Autor:
Maria do Carmo Costa, Fernanda Bajanca, Ana-João Rodrigues, Ricardo J Tomé, Garry Corthals, Sandra Macedo-Ribeiro, Henry L Paulson, Elsa Logarinho, Patrícia Maciel
Publikováno v:
PLoS ONE, Vol 5, Iss 7, p e11728 (2010)
During myogenesis several transcription factors and regulators of protein synthesis and assembly are rapidly degraded by the ubiquitin-proteasome system (UPS). Given the potential role of the deubiquitinating enzyme (DUB) ataxin-3 in the UPS, and the
Externí odkaz:
https://doaj.org/article/d6c7b0009fe141218f443670c1c55bf3