Glutamine repeats and neurodegeneration
A growing number of neurodegenerative diseases have been found to result from the
expansion of an unstable trinucleotide repeat. Over the past 6 years, researchers have …
expansion of an unstable trinucleotide repeat. Over the past 6 years, researchers have …
Trinucleotide repeats: mechanisms and pathophysiology
CJ Cummings, HY Zoghbi - Annual review of genomics and …, 2000 - annualreviews.org
Within the closing decade of the twentieth century, 14 neurological disorders were shown to
result from the expansion of unstable trinucleotide repeats, establishing this once unique …
result from the expansion of unstable trinucleotide repeats, establishing this once unique …
Huntingtin expression stimulates endosomal–lysosomal activity, endosome tubulation, and autophagy
KB Kegel, M Kim, E Sapp, C McIntyre… - Journal of …, 2000 - Soc Neuroscience
An expansion of polyglutamines in the N terminus of huntingtin causes Huntington's disease
(HD) and results in the accrual of mutant protein in the nucleus and cytoplasm of affected …
(HD) and results in the accrual of mutant protein in the nucleus and cytoplasm of affected …
Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease
M Turmaine, A Raza, A Mahal… - Proceedings of the …, 2000 - National Acad Sciences
Huntington's disease (HD) is a fatal inherited neurodegenerative disorder characterized by
personality changes, motor impairment, and subcortical dementia. HD is one of a number of …
personality changes, motor impairment, and subcortical dementia. HD is one of a number of …
Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human
DP Huynh, K Figueroa, N Hoang, SM Pulst - Nature genetics, 2000 - nature.com
Instability of CAG DNA trinucleotide repeats is the mutational mechanism for several
neurodegenerative diseases resulting in the expansion of a polyglutamine (polyQ) tract …
neurodegenerative diseases resulting in the expansion of a polyglutamine (polyQ) tract …
Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease
A Wyttenbach, J Carmichael, J Swartz… - Proceedings of the …, 2000 - National Acad Sciences
Huntington's disease (HD), spinocerebellar ataxias types 1 and 3 (SCA1, SCA3), and
spinobulbar muscular atrophy (SBMA) are caused by CAG/polyglutamine expansion …
spinobulbar muscular atrophy (SBMA) are caused by CAG/polyglutamine expansion …
Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription
T Shimohata, T Nakajima, M Yamada, C Uchida… - Nature …, 2000 - nature.com
At least eight inherited neurodegenerative diseases are caused by expanded CAG repeats
encoding polyglutamine (polyQ) stretches. Although cytotoxicities of expanded polyQ …
encoding polyglutamine (polyQ) stretches. Although cytotoxicities of expanded polyQ …
Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila
HYE Chan, JM Warrick, GL Gray-Board… - Human molecular …, 2000 - academic.oup.com
At least eight dominant human neurodegenerative diseases are due to the expansion of a
polyglutamine within the disease proteins. This confers toxicity on the proteins and is …
polyglutamine within the disease proteins. This confers toxicity on the proteins and is …
The ubiquitin‐mediated proteolytic pathway: Mode of action and clinical implications
A Ciechanover, A Orian… - Journal of cellular …, 2000 - Wiley Online Library
Proteolysis via the ubiquitin system plays important roles in a variety of basic cellular
processes. Among these are regulation of cell cycle and division, modulation of the immune …
processes. Among these are regulation of cell cycle and division, modulation of the immune …
A novel protein with RNA-binding motifs interacts with ataxin-2
H Shibata, DP Huynh, SM Pulst - Human molecular genetics, 2000 - academic.oup.com
Abstract Spinocerebellar ataxia type 2 (SCA2) is caused by expansion of a polyglutamine
tract in ataxin-2, a protein of unknown function. Using the yeast two-hybrid system, we …
tract in ataxin-2, a protein of unknown function. Using the yeast two-hybrid system, we …