Zobrazeno 1 - 10
of 10
pro vyhledávání: '"Isabella A. Lambert-Smith"'
Autor:
Isabella A. Lambert-Smith, Victoria K. Shephard, Luke McAlary, Justin J. Yerbury, Darren N. Saunders
Publikováno v:
Scientific Reports, Vol 14, Iss 1, Pp 1-11 (2024)
Abstract Disrupted proteome homeostasis (proteostasis) in amyotrophic lateral sclerosis (ALS) has been a major focus of research in the past two decades. However, the proteostasis processes that become disturbed in ALS are not fully understood. Obtai
Externí odkaz:
https://doaj.org/article/c51f8b64ed1441c5bf5d02c1a1968d68
Disrupted proteome homeostasis (proteostasis) in amyotrophic lateral sclerosis (ALS) has been a major focus of research in the past two decades. Yet the exact processes that normally maintain proteostasis, but that are uniquely disturbed in motor neu
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::ab39fba112b8746ad86ae4ad970abbfa
https://doi.org/10.1101/2021.09.23.461468
https://doi.org/10.1101/2021.09.23.461468
Publikováno v:
Progress in biophysics and molecular biology. 174
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive and fatal neurodegenerative disease that results from the loss of both upper and lower motor neurons. It is the most common motor neuron disease and currently has no effective treatment. Th
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::51f14824a15166a8b0c3d908cf45a4db
https://pubmed.ncbi.nlm.nih.gov/35716729
https://pubmed.ncbi.nlm.nih.gov/35716729
Publikováno v:
The international journal of biochemistrycell biology. 123
Ubiquitin-activating enzyme E1, UBA1, functions at the apex of the enzymatic ubiquitylation cascade, catalysing ubiquitin activation. UBA1 is thus of fundamental importance to the modulation of ubiquitin homeostasis and to all downstream ubiquitylati
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2b4858550eb51d54b6a25f2b6f1c031d
https://pubmed.ncbi.nlm.nih.gov/32315770
https://pubmed.ncbi.nlm.nih.gov/32315770
Autor:
Kara L. Vine, Jessie McKenna, Prajwal Ciryam, Isabella A. Lambert-Smith, Kristen Mitchell, Natalie E. Farrawell, Darren N. Saunders, Justin J. Yerbury, Luke McAlary
Publikováno v:
Journal of Cell Science.
A hallmark of amyotrophic lateral sclerosis (ALS) pathology is the accumulation of ubiquitylated protein inclusions within motor neurons. Recent studies suggest the sequestration of ubiquitin (Ub) into inclusions reduces the availability of free Ub,
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::a4d5c9803c0470e8a49e1f3d232e6db4
https://doi.org/10.1242/jcs.209122
https://doi.org/10.1242/jcs.209122
Autor:
Natalie E. Farrawell, Isabella A. Lambert-Smith, Kristen Mitchell, Prajwal Ciryam, Jessie McKenna, Kara L. Vine, Justin J. Yerbury, Darren N. Saunders, Luke McAlary
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease involving the selective death of upper and lower motor neurons in the primary motor cortex and spinal cord. A hallmark of ALS pathology is the accumulation of ubiquitinated prot
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5223c3a45375e56ba3d8fd08c24a4fc9
ALS is characterised by a focal onset of motor neuron loss, followed by contiguous outward spreading of pathology throughout the nervous system, resulting in paralysis and death generally within a few years after diagnosis. The aberrant release and u
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9dde11a35b5657320e58b12100acc232
https://europepmc.org/articles/PMC5655364/
https://europepmc.org/articles/PMC5655364/
Autor:
Isabella A. Lambert-Smith, Stephen G. Oliver, Giorgio Favrin, Gian Gaetano Tartaglia, Christopher M. Dobson, Mark R. Wilson, Fernando Cid, Richard I. Morimoto, Justin J. Yerbury, Rosie Freer, Darren N. Saunders, Prajwal Ciryam, Daniel Bean, Michele Vendruscolo
Publikováno v:
Proceedings of the National Academy of Sciences. 114
Amyotrophic lateral sclerosis (ALS) is a heterogeneous degenerative motor neuron disease linked to numerous genetic mutations in apparently unrelated proteins. These proteins, including SOD1, TDP-43, and FUS, are highly aggregation-prone and form a v
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7a2ae526a29e41b313249569a23d2411
https://doi.org/10.1073/pnas.1613854114
https://doi.org/10.1073/pnas.1613854114
Autor:
Isabella A. Lambert-Smith, Justin J. Yerbury, Ian P. Blair, Darren N. Saunders, Natalie E. Farrawell, Sadaf T. Warraich, Danny M. Hatters
Publikováno v:
Scientific Reports
Amyotrophic lateral sclerosis is a rapidly progressing neurodegenerative disease associated with protein misfolding and aggregation. Most cases are characterized by TDP-43 positive inclusions, while a minority of familial ALS cases are instead FUS an
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::60fe6334e7c644dc8c376fa5483ee9b0
http://europepmc.org/articles/PMC4544019
http://europepmc.org/articles/PMC4544019
Publikováno v:
Amyotrophic lateral sclerosisfrontotemporal degeneration. 15
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fa2e46b33566595c135c27d5adb2dfa2
https://pubmed.ncbi.nlm.nih.gov/25382839
https://pubmed.ncbi.nlm.nih.gov/25382839