[HTML][HTML] Endoplasmic reticulum stress: cell life and death decisions
C Xu, B Bailly-Maitre, JC Reed - The Journal of clinical …, 2005 - Am Soc Clin Investig
Disturbances in the normal functions of the ER lead to an evolutionarily conserved cell
stress response, the unfolded protein response, which is aimed initially at compensating for …
stress response, the unfolded protein response, which is aimed initially at compensating for …
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 …
Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
E Scherzinger, R Lurz, M Turmaine, L Mangiarini… - Cell, 1997 - cell.com
The mechanism by which an elongated polyglutamine sequence causes neurodegeneration
in Huntington's disease (HD) is unknown. In this study, we show that the proteolytic cleavage …
in Huntington's disease (HD) is unknown. In this study, we show that the proteolytic cleavage …
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology
The data we report in this study concern the types, location, numbers, forms, and
composition of microscopic huntingtin aggregates in brain tissues from humans with different …
composition of microscopic huntingtin aggregates in brain tissues from humans with different …
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
JG Hodgson, N Agopyan, CA Gutekunst, BR Leavitt… - Neuron, 1999 - cell.com
We have produced yeast artificial chromosome (YAC) transgenic mice expressing normal
(YAC18) and mutant (YAC46 and YAC72) huntingtin (htt) in a developmental and tissue …
(YAC18) and mutant (YAC46 and YAC72) huntingtin (htt) in a developmental and tissue …
Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3
The mechanism of neurodegeneration in CAG/polyglutamine repeat expansion diseases is
unknown but is thought to occur at the protein level. Here, in studies of spinocerebellar …
unknown but is thought to occur at the protein level. Here, in studies of spinocerebellar …
Molecular pathways to neurodegeneration
The molecular bases underlying the pathogenesis of neurodegenerative diseases are
gradually being disclosed. One problem that investigators face is distinguishing primary from …
gradually being disclosed. One problem that investigators face is distinguishing primary from …
Slowing of neurodegeneration in Parkinson's disease and Huntington's disease: future therapeutic perspectives
Several important advances have been made in our understanding of the pathways that
lead to cell dysfunction and death in Parkinson's disease and Huntington's disease. These …
lead to cell dysfunction and death in Parkinson's disease and Huntington's disease. These …
Molecular pathophysiological mechanisms in Huntington's disease
A Jurcau - Biomedicines, 2022 - mdpi.com
Huntington's disease is an inherited neurodegenerative disease described 150 years ago
by George Huntington. The genetic defect was identified in 1993 to be an expanded CAG …
by George Huntington. The genetic defect was identified in 1993 to be an expanded CAG …
Dominant phenotypes produced by the HD mutation in STHdhQ111 striatal cells
F Trettel, D Rigamonti, P Hilditch-Maguire… - Human molecular …, 2000 - academic.oup.com
Lengthening a glutamine tract in huntingtin confers a dominant attribute that initiates
degeneration of striatal neurons in Huntington's disease (HD). To identify pathways that are …
degeneration of striatal neurons in Huntington's disease (HD). To identify pathways that are …