The protective role of exercise against age-related neurodegeneration

A Sujkowski, L Hong, RJ Wessells, SV Todi - Ageing Research Reviews, 2022 - Elsevier
Endurance exercise is a widely accessible, low-cost intervention with a variety of benefits to
multiple organ systems. Exercise improves multiple indices of physical performance and …

Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis

L Schöls, P Bauer, T Schmidt, T Schulte… - The Lancet …, 2004 - thelancet.com
Autosomal dominant cerebellar ataxias are hereditary neurodegenerative disorders that are
known as spinocerebellar ataxias (SCA) in genetic nomenclature. In the pregenomic era …

Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

M Tanaka, Y Machida, S Niu, T Ikeda, NR Jana… - Nature medicine, 2004 - nature.com
Inhibition of polyglutamine-induced protein aggregation could provide treatment options for
polyglutamine diseases such as Huntington disease. Here we showed through in vitro …

Self-assembly of polyglutamine-containing huntingtin fragments into amyloid-like fibrils: implications for Huntington's disease pathology

E Scherzinger, A Sittler, K Schweiger… - Proceedings of the …, 1999 - National Acad Sciences
Huntington's disease is a progressive neurodegenerative disorder caused by a
polyglutamine [poly (Q)] repeat expansion in the first exon of the huntingtin protein …

Progressive disruption of cellular protein folding in models of polyglutamine diseases

T Gidalevitz, A Ben-Zvi, KH Ho, HR Brignull… - Science, 2006 - science.org
Numerous human diseases are associated with the chronic expression of misfolded and
aggregation-prone proteins. The expansion of polyglutamine residues in unrelated proteins …

Huntingtin–protein interactions and the pathogenesis of Huntington's disease

SH Li, XJ Li - TRENDS in Genetics, 2004 - cell.com
At least nine inherited neurodegenerative diseases share a polyglutamine expansion in
their respective disease proteins. These diseases show distinct neuropathological changes …

[HTML][HTML] Disruption of axonal transport by loss of huntingtin or expression of pathogenic polyQ proteins in Drosophila

S Gunawardena, LS Her, RG Brusch, RA Laymon… - Neuron, 2003 - cell.com
We tested whether proteins implicated in Huntington's and other polyglutamine (polyQ)
expansion diseases can cause axonal transport defects. Reduction of Drosophila huntingtin …

SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein

K Nakamura, SY Jeong, T Uchihara… - Human molecular …, 2001 - academic.oup.com
Genetic etiologies of at least 20% of autosomal dominant cerebellar ataxias (ADCAs) have
yet to be clarified. We identified a novel spinocerebellar ataxia (SCA) form in four Japanese …

Toward understanding Machado–Joseph disease

M do Carmo Costa, HL Paulson - Progress in neurobiology, 2012 - Elsevier
Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is
the most common inherited spinocerebellar ataxia and one of many polyglutamine …

Huntingtin interacting proteins are genetic modifiers of neurodegeneration

LS Kaltenbach, E Romero, RR Becklin, R Chettier… - PLoS …, 2007 - journals.plos.org
Huntington's disease (HD) is a fatal neurodegenerative condition caused by expansion of
the polyglutamine tract in the huntingtin (Htt) protein. Neuronal toxicity in HD is thought to be …