Huntington's disease: from molecular pathogenesis to clinical treatment

CA Ross, SJ Tabrizi - The Lancet Neurology, 2011 - thelancet.com
Huntington's disease is a progressive, fatal, neurodegenerative disorder caused by an
expanded CAG repeat in the huntingtin gene, which encodes an abnormally long …

Small heat shock proteins, big impact on protein aggregation in neurodegenerative disease

JM Webster, AL Darling, VN Uversky… - Frontiers in …, 2019 - frontiersin.org
Misfolding, aggregation, and aberrant accumulation of proteins are central components in
the progression of neurodegenerative disease. Cellular molecular chaperone systems …

Neuronal aggregates: formation, clearance, and spreading

J Lim, Z Yue - Developmental cell, 2015 - cell.com
Proteostasis is maintained by multiple cellular pathways, including protein synthesis, quality
control, and degradation. An imbalance of neuronal proteostasis, associated with protein …

Heat shock proteins: cellular and molecular mechanisms in the central nervous system

RA Stetler, Y Gan, W Zhang, AK Liou, Y Gao… - Progress in …, 2010 - Elsevier
Emerging evidence indicates that heat shock proteins (HSPs) are critical regulators in
normal neural physiological function as well as in cell stress responses. The functions of …

Huntington's disease: from pathology and genetics to potential therapies

S Imarisio, J Carmichael, V Korolchuk… - Biochemical …, 2008 - portlandpress.com
Huntington's disease (HD) is a devastating autosomal dominant neurodegenerative disease
caused by a CAG trinucleotide repeat expansion encoding an abnormally long …

[HTML][HTML] Mitochondria in Huntington's disease

M Damiano, L Galvan, N Déglon, E Brouillet - Biochimica et Biophysica …, 2010 - Elsevier
Huntington's disease (HD) is an inherited progressive neurodegenerative disorder
associated with involuntary abnormal movements (chorea), cognitive deficits and psychiatric …

The S/T-rich motif in the DNAJB6 chaperone delays polyglutamine aggregation and the onset of disease in a mouse model

V Kakkar, C Månsson, EP de Mattos, S Bergink… - Molecular cell, 2016 - cell.com
Expanded CAG repeats lead to debilitating neurodegenerative disorders characterized by
aggregation of proteins with expanded polyglutamine (polyQ) tracts. The mechanism of …

HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration

M Mielcarek, C Landles, A Weiss, A Bradaia… - PLoS …, 2013 - journals.plos.org
Histone deacetylase (HDAC) 4 is a transcriptional repressor that contains a glutamine-rich
domain. We hypothesised that it may be involved in the molecular pathogenesis of …

[HTML][HTML] Small heat shock proteins in neurodegenerative diseases

L Vendredy, E Adriaenssens, V Timmerman - Cell Stress and Chaperones, 2020 - Elsevier
Small heat shock proteins are ubiquitously expressed chaperones, yet mutations in some of
them cause tissue-specific diseases. Here, we will discuss how small heat shock proteins …

Protein homeostasis in models of aging and age-related conformational disease

EA Kikis, T Gidalevitz, RI Morimoto - Protein Metabolism and Homeostasis …, 2010 - Springer
T he stability of the proteome is crucial to the health of the cell, and contributes significantly
to the lifespan of the organism. Aging and many age-related diseases have in common the …