A Kasumu, I Bezprozvanny - The Cerebellum, 2012 - Springer
Spinocerebellar ataxias (SCAs) constitute a heterogeneous group of more than 30 autosomal-dominant genetic and neurodegenerative disorders. SCAs are generally …
J Liu, TS Tang, H Tu, O Nelson, E Herndon… - Journal of …, 2009 - Soc Neuroscience
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominantly inherited, neurodegenerative disease caused by an expansion of polyglutamine tracts in the cytosolic …
R Hourez, L Servais, D Orduz, D Gall… - Journal of …, 2011 - Soc Neuroscience
The contribution of neuronal dysfunction to neurodegeneration is studied in a mouse model of spinocerebellar ataxia type 1 (SCA1) displaying impaired motor performance ahead of …
Neuronal atrophy in neurodegenerative diseases is commonly viewed as an early event in a continuum that ultimately results in neuronal loss. In a mouse model of the polyglutamine …
HG Serra, CE Byam, JD Lande… - Human molecular …, 2004 - academic.oup.com
Abstract Spinocerebellar ataxia type 1 (SCA1) is a neurodegenerative disease caused by the expansion of a polyglutamine repeat within the disease protein, ataxin 1. To elucidate …
Neurodegenerative diseases (NDDs) involve years of gradual preclinical progression. It is widely anticipated that in order to be effective, treatments should target early stages of …
Abstract Spinocerebellar ataxia type 6 (SCA6) is a devastating midlife-onset autosomal dominant motor control disease with no known treatment. Using a hyper-expanded …
KJ Robinson, M Watchon, AS Laird - Frontiers in neuroscience, 2020 - frontiersin.org
The spinocerebellar ataxias (SCAs) are a heterogeneous group of neurodegenerative diseases that share convergent disease features. A common symptom of these diseases is …
OA Andreassen, A Dedeoglu, V Stanojevic… - Neurobiology of …, 2002 - Elsevier
In a transgenic mouse model of the neurodegenerative disorder Huntington's disease (HD), age-dependent neurologic defects are accompanied by progressive alterations in glucose …