Glutamine repeats and neurodegeneration

HY Zoghbi, HT Orr - Annual review of neuroscience, 2000 - annualreviews.org
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 …

Targeting Hsp90/Hsp70-based protein quality control for treatment of adult onset neurodegenerative diseases

WB Pratt, JE Gestwicki, Y Osawa… - Annual review of …, 2015 - annualreviews.org
Currently available therapies for adult onset neurodegenerative diseases provide
symptomatic relief but do not modify disease progression. Here we explore a new …

Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein

JP Taylor, F Tanaka, J Robitschek… - Human molecular …, 2003 - academic.oup.com
Expression of misfolded protein in cultured cells frequently leads to the formation of
juxtanuclear inclusions that have been termed 'aggresomes'. Aggresome formation is an …

CREB-binding protein sequestration by expanded polyglutamine

A McCampbell, JP Taylor, AA Taye… - Human molecular …, 2000 - academic.oup.com
Spinal and bulbar muscular atrophy (SBMA) is one of eight inherited neurodegenerative
diseases known to be caused by CAG repeat expansion. The expansion results in an …

Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the …

DL Stenoien, CJ Cummings, HP Adams… - Human molecular …, 1999 - academic.oup.com
Spinal bulbar muscular atrophy is a neurodegenerative disorder caused by a polyglutamine
expansion in the androgen receptor (AR). We show in transiently transfected HeLa cells that …

Glutamine repeats and neurodegenerative diseases: molecular aspects

MF Perutz - Trends in biochemical sciences, 1999 - cell.com
Eight severe inherited neurodegenerative diseases are caused by expansion of glutamine
repeats in the affected proteins. In every case, proteins with repeats of fewer than 38 …

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 …

[HTML][HTML] Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport

G Szebenyi, GA Morfini, A Babcock, M Gould, K Selkoe… - Neuron, 2003 - cell.com
Huntington's and Kennedy's disease are autosomal dominant neurodegenerative diseases
caused by pathogenic expansion of polyglutamine tracts. Expansion of glutamine repeats …

Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation

MK Perez, HL Paulson, SJ Pendse, SJ Saionz… - The Journal of cell …, 1998 - rupress.org
The inherited neurodegenerative diseases caused by an expanded glutamine repeat share
the pathologic feature of intranuclear aggregates or inclusions (NI). Here in cell-based …

Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins

CC Huang, PW Faber, F Persichetti, V Mittal… - Somatic cell and …, 1998 - Springer
Huntington's disease (HD) is caused by an expanded CAG trinucleotide repeat encoding a
tract of consecutive glutamines near the amino terminus of huntingtin, a large protein of …