TY - JOUR
T1 - Multiple system atrophy
T2 - A clinical and neuropathological perspective
AU - Ubhi, Kiren
AU - Low, Phillip
AU - Masliah, Eliezer
N1 - Funding Information:
Our work is supported by the National Institutes of Health (NS044233, AG18440, AG022074) and by the Donner Canadian Foundation.
PY - 2011/11
Y1 - 2011/11
N2 - Multiple system atrophy (MSA) is a neurodegenerative disease involving motor abnormalities that include akinesia, rigidity and postural instability. While improved diagnostic criteria have aided the accurate diagnosis of MSA, our understanding of the neuropathological aspects underlying MSA was bolstered by the identification of α-synuclein (α-syn) as the primary constituent of the abnormal protein aggregates observed in the brains of MSA patients. The generation of transgenic animal models of MSA coupled with an increasing understanding of the biochemical structure and function of α-syn has highlighted a number of key pathological pathways thought to underlie the neurodegeneration observed in MSA. This review summarizes key findings in the field, discusses current areas of debate, and describes current experimental approaches towards disease-modifying therapies.
AB - Multiple system atrophy (MSA) is a neurodegenerative disease involving motor abnormalities that include akinesia, rigidity and postural instability. While improved diagnostic criteria have aided the accurate diagnosis of MSA, our understanding of the neuropathological aspects underlying MSA was bolstered by the identification of α-synuclein (α-syn) as the primary constituent of the abnormal protein aggregates observed in the brains of MSA patients. The generation of transgenic animal models of MSA coupled with an increasing understanding of the biochemical structure and function of α-syn has highlighted a number of key pathological pathways thought to underlie the neurodegeneration observed in MSA. This review summarizes key findings in the field, discusses current areas of debate, and describes current experimental approaches towards disease-modifying therapies.
UR - http://www.scopus.com/inward/record.url?scp=82455205801&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=82455205801&partnerID=8YFLogxK
U2 - 10.1016/j.tins.2011.08.003
DO - 10.1016/j.tins.2011.08.003
M3 - Review article
C2 - 21962754
AN - SCOPUS:82455205801
SN - 0166-2236
VL - 34
SP - 581
EP - 590
JO - Trends in neurosciences
JF - Trends in neurosciences
IS - 11
ER -