The unfolded protein response, degradation from the endoplasmic reticulum, and cancer
YC Tsai, AM Weissman - Genes & cancer, 2010 - journals.sagepub.com
The endoplasmic reticulum (ER) is an essential organelle involved in many cellular
functions including protein folding and secretion, lipid biosynthesis, and calcium …
functions including protein folding and secretion, lipid biosynthesis, and calcium …
The AAA+ ATPase p97, a cellular multitool
L Stach, PS Freemont - Biochemical Journal, 2017 - portlandpress.com
The AAA+ (ATPases associated with diverse cellular activities) ATPase p97 is essential to a
wide range of cellular functions, including endoplasmic reticulum-associated degradation …
wide range of cellular functions, including endoplasmic reticulum-associated degradation …
Machado–Joseph disease/spinocerebellar ataxia type 3: lessons from disease pathogenesis and clues into therapy
CA Matos, LP de Almeida… - Journal of …, 2019 - Wiley Online Library
Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA 3), is an incurable disorder, widely regarded as the most common form of …
(SCA 3), is an incurable disorder, widely regarded as the most common form of …
Impaired ERAD and ER stress are early and specific events in polyglutamine toxicity
ML Duennwald, S Lindquist - Genes & development, 2008 - genesdev.cshlp.org
Protein misfolding, whether caused by aging, environmental factors, or genetic mutations, is
a common basis for neurodegenerative diseases. The misfolding of proteins with abnormally …
a common basis for neurodegenerative diseases. The misfolding of proteins with abnormally …
The spinocerebellar ataxias
HL Paulson - Journal of neuro-ophthalmology, 2009 - journals.lww.com
Slowly progressive ataxia accompanied by cerebellar degeneration is often genetic in
origin. The past 15 years have witnessed a revolution in our understanding of the causes of …
origin. The past 15 years have witnessed a revolution in our understanding of the causes of …
Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3
FM Menzies, J Huebener, M Renna, M Bonin, O Riess… - Brain, 2010 - academic.oup.com
Spinocerebellar ataxia type 3 is a neurodegenerative disorder caused by the expansion of
the polyglutamine repeat region within the ataxin-3 protein. The mutant protein forms …
the polyglutamine repeat region within the ataxin-3 protein. The mutant protein forms …
The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains
BJ Winborn, SM Travis, SV Todi, KM Scaglione… - Journal of Biological …, 2008 - ASBMB
Ubiquitin chain complexity in cells is likely regulated by a diverse set of deubiquitinating
enzymes (DUBs) with distinct ubiquitin chain preferences. Here we show that the …
enzymes (DUBs) with distinct ubiquitin chain preferences. Here we show that the …
The otubain YOD1 is a deubiquitinating enzyme that associates with p97 to facilitate protein dislocation from the ER
YOD1 is a highly conserved deubiquitinating enzyme of the ovarian tumor (otubain) family,
whose function has yet to be assigned in mammalian cells. YOD1 is a constituent of a …
whose function has yet to be assigned in mammalian cells. YOD1 is a constituent of a …
Deubiquitinases and the new therapeutic opportunities offered to cancer
R Pfoh, IK Lacdao, V Saridakis - Endocrine-related cancer, 2015 - erc.bioscientifica.com
Deubiquitinases (DUBs) play important roles and therefore are potential drug targets in
various diseases including cancer and neurodegeneration. In this review, we recapitulate …
various diseases including cancer and neurodegeneration. In this review, we recapitulate …
[HTML][HTML] The Cdc48 machine in endoplasmic reticulum associated protein degradation
DH Wolf, A Stolz - Biochimica et Biophysica Acta (BBA)-Molecular Cell …, 2012 - Elsevier
The AAA-type ATPase Cdc48 (named p97/VCP in mammals) is a molecular machine in all
eukaryotic cells that transforms ATP hydrolysis into mechanic power to unfold and pull …
eukaryotic cells that transforms ATP hydrolysis into mechanic power to unfold and pull …