TY - JOUR
T1 - The ins and outs of a polyglutamine neurodegenerative disease
T2 - Spinocerebellar ataxia type 1 (SCA1)
AU - Orr, Harry T.
PY - 2000/6
Y1 - 2000/6
N2 - Polyglutamine neurodegenerative disorders are characterized by the expansion of a glutamine tract within the mutant disease-causing protein. Expression of the mutant protein induces a progressive loss of neuronal function and the subsequent neurodegeneration of a set of neurons characteristic to each disease. Spinocerebellar ataxia type 1 (SCA1) is one polyglutamine disease where various experimental model systems, in particular transgenic mice, have been utilized to dissect the molecular and cellular events important for disease. This review summarizes these findings and places them in a context of potential future research directions. (C) 2000 Academic Press.
AB - Polyglutamine neurodegenerative disorders are characterized by the expansion of a glutamine tract within the mutant disease-causing protein. Expression of the mutant protein induces a progressive loss of neuronal function and the subsequent neurodegeneration of a set of neurons characteristic to each disease. Spinocerebellar ataxia type 1 (SCA1) is one polyglutamine disease where various experimental model systems, in particular transgenic mice, have been utilized to dissect the molecular and cellular events important for disease. This review summarizes these findings and places them in a context of potential future research directions. (C) 2000 Academic Press.
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U2 - 10.1006/nbdi.2000.0305
DO - 10.1006/nbdi.2000.0305
M3 - Review article
C2 - 10860780
AN - SCOPUS:0034210773
SN - 0969-9961
VL - 7
SP - 129
EP - 134
JO - Neurobiology of Disease
JF - Neurobiology of Disease
IS - 3
ER -