TY - JOUR
T1 - Polyglutamine diseases
T2 - Protein cleavage and aggregation
AU - Zoghbi, Huda Y.
AU - Orr, Harry T.
PY - 1999/10/1
Y1 - 1999/10/1
N2 - Neuronal aggregates of the disease-causing protein, often in the nucleus of affected cells, are a pathological hallmark of the neurodegenerative diseases known as polyglutamine disorders. It was suggested that these nuclear aggregates are the cause of these disorders. However, recent evidence suggests that the aggregates, in fact, are not the pathogenic basis and, instead, may play a role in sequestration of the pathogenic protein.
AB - Neuronal aggregates of the disease-causing protein, often in the nucleus of affected cells, are a pathological hallmark of the neurodegenerative diseases known as polyglutamine disorders. It was suggested that these nuclear aggregates are the cause of these disorders. However, recent evidence suggests that the aggregates, in fact, are not the pathogenic basis and, instead, may play a role in sequestration of the pathogenic protein.
UR - http://www.scopus.com/inward/record.url?scp=0032824836&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0032824836&partnerID=8YFLogxK
U2 - 10.1016/S0959-4388(99)00013-6
DO - 10.1016/S0959-4388(99)00013-6
M3 - Review article
C2 - 10508741
AN - SCOPUS:0032824836
SN - 0959-4388
VL - 9
SP - 566
EP - 570
JO - Current opinion in neurobiology
JF - Current opinion in neurobiology
IS - 5
ER -