Results 181 to 190 of about 16,136 (210)

GABAergic neuronal dysfunction underlies tremor in spinocerebellar ataxia 3.

Dis Model Mech
Banerjee A, Chatterjee M, Tan KJ, Tay S, Duan K, Andiappan AK, Howland SW, Aso Y, Aw SS.   +8 more
europepmc   +1 more source

Conserved Aberrant Developmental Trajectories of Human and Mouse SBMA Motor Neurons

Devine H, Roberts MJ, Ziff OJ, Hanna MG, Greensmith L, Patani R, Malik B.   +6 more
europepmc   +1 more source

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Autophagy and Polyglutamine Disease

2020
Polyglutamine (polyQ) disease is a type of fatal neurodegenerative disease caused by an expansion of CAG repeats in a specific gene, resulting in a protein with an abnormal polyQ fragment. The age of onset and the degree of pathological deterioration are related to the length of the polyQ fragment.
Haigang, Ren, Zongbing, Hao, Guanghui, Wang   +2 more
openaire   +2 more sources

Proteomics of Polyglutamine Aggregates

2006
In nine members of polyglutamine (polyQ) diseases, CAG repeat expansions of their responsible genes are observed. The disease is considered to be caused by the formation of polyQ aggregates that sequester proteins essential for cell viability. To understand the pathological process of polyQ diseases, a proteomic approach was used to identify aggregate ...
Kenichi, Mitsui, Hiroshi, Doi, Nobuyuki, Nukina   +2 more
openaire   +2 more sources

Folding of polyglutamine chains

The Journal of Chemical Physics, 2008
Long polyglutamine chains have been associated with a number of neurodegenerative diseases. These include Huntington’s disease, where expanded polyglutamine (PolyQ) sequences longer than 36 residues are correlated with the onset of symptoms. In this paper we study the folding pathway of a 54-residue PolyQ chain into a β-helical structure.
Manan, Chopra, Allam S, Reddy, N L, Abbott, J J, de Pablo   +3 more
openaire   +2 more sources

Cell death in polyglutamine diseases

Cell and Tissue Research, 2000
An increasing number of inherited neurodegenerative diseases are known to be caused by trinucleotide repeat expansions in the respective genes. At least nine disorders result from a CAG trinucleotide repeat expansion which is translated into a polyglutamine stretch in the respective proteins: Huntington's disease (HD), dentatorubral pallidolysian ...
B O, Evert, U, Wüllner, T, Klockgether
openaire   +2 more sources