SCA3: neurological features, pathogenesis and animal models
O Riess, U Rüb, A Pastore, P Bauer, L Schöls - The Cerebellum, 2008 - Springer
The most frequent subtype of autosomal dominant inherited spinocerebellar ataxias is
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …
caused by CAG repeat expansions of more than 55 units in the ataxin-3 gene. The clinical …
Aggresome formation and neurodegenerative diseases: therapeutic implications
LS Chin, JA Olzmann, L Li - Current medicinal chemistry, 2008 - ingentaconnect.com
Accumulation of misfolded proteins in proteinaceous inclusions is a prominent pathological
feature common to many agerelated neurodegenerative diseases, including Parkinson's …
feature common to many agerelated neurodegenerative diseases, including Parkinson's …
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 …
Deranged calcium signaling and neurodegeneration in spinocerebellar ataxia type 3
X Chen, TS Tang, H Tu, O Nelson, M Pook… - Journal of …, 2008 - Soc Neuroscience
Spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease (MJD), is
an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion …
an autosomal-dominant neurodegenerative disorder caused by a polyglutamine expansion …
Inhibition of p97-dependent protein degradation by Eeyarestatin I
Q Wang, L Li, Y Ye - Journal of Biological Chemistry, 2008 - ASBMB
Elimination of misfolded proteins from the endoplasmic reticulum (ER) by ER-associated
degradation involves substrate retrotranslocation from the ER lumen into the cytosol for …
degradation involves substrate retrotranslocation from the ER lumen into the cytosol for …
New insights into the pathoanatomy of spinocerebellar ataxia type 3 (Machado–Joseph disease)
U Rüb, ER Brunt, T Deller - Current opinion in neurology, 2008 - journals.lww.com
Systematic pathoanatomical analysis of spinocerebellar ataxia type 3 brains helps to
understand the structural basis of this neurodegenerative disease and offers explanations …
understand the structural basis of this neurodegenerative disease and offers explanations …
Polyglutamine-expanded ataxin-3 causes cerebellar dysfunction of SCA3 transgenic mice by inducing transcriptional dysregulation
In the present study, we prepared a SCA3 animal model by generating transgenic mice
expressing polyglutamine-expanded ataxin-3-Q79. Ataxin-3-Q79 was expressed in brain …
expressing polyglutamine-expanded ataxin-3-Q79. Ataxin-3-Q79 was expressed in brain …
Functional characterization of the Arabidopsis ubiquitin‐specific protease gene family reveals specific role and redundancy of individual members in development
Y Liu, F Wang, H Zhang, H He, L Ma… - The Plant …, 2008 - Wiley Online Library
Ubiquitin‐specific proteases (UBPs) are a highly conserved family of proteins in eukaryotes,
and play critical roles in protein de‐ubiquitination. Here we report a systematic genetic and …
and play critical roles in protein de‐ubiquitination. Here we report a systematic genetic and …
Deciphering the ubiquitin-mediated pathway in apicomplexan parasites: a potential strategy to interfere with parasite virulence
N Ponts, J Yang, DWD Chung, J Prudhomme, T Girke… - PloS one, 2008 - journals.plos.org
Background Reversible modification of proteins through the attachment of ubiquitin or
ubiquitin-like modifiers is an essential post-translational regulatory mechanism in …
ubiquitin-like modifiers is an essential post-translational regulatory mechanism in …
Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease
S Alves, E Régulier… - Human molecular …, 2008 - academic.oup.com
Machado-Joseph disease (MJD) is a fatal, dominant neurodegenerative disorder. MJD
results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of …
results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of …