TY - JOUR
T1 - A close association of torsinA and α-synuclein in lewy bodies
T2 - A fluorescence resonance energy transfer study
AU - Sharma, Nutan
AU - Hewett, Jeffrey
AU - Ozelius, Laurie J.
AU - Ramesh, Vijaya
AU - McLean, Pamela J.
AU - Breakefield, Xandra O.
AU - Hyman, Bradley T.
PY - 2001/7
Y1 - 2001/7
N2 - TorsinA, a novel protein in which a mutation causes dominant, early onset torsion dystonia, may serve as a chaperone for misfolded proteins that require refolding or degradation. It has been hypothesized that misfolded α-synuclein, a protein in which two mutations cause autosomal dominantly inherited Parkinson's disease, serves as a nidus for the development of a Lewy body. We hypothesized that torsinA plays a role in the cellular processing of α-synuclein. We demonstrate that anti-torsin antibodies stain Lewy bodies and Lewy neurites in the substantia nigra and cortex. Using sensitive fluorescent resonance energy transfer (FRET) techniques, we find evidence of a close association between torsinA and α-synuclein in Lewy bodies.
AB - TorsinA, a novel protein in which a mutation causes dominant, early onset torsion dystonia, may serve as a chaperone for misfolded proteins that require refolding or degradation. It has been hypothesized that misfolded α-synuclein, a protein in which two mutations cause autosomal dominantly inherited Parkinson's disease, serves as a nidus for the development of a Lewy body. We hypothesized that torsinA plays a role in the cellular processing of α-synuclein. We demonstrate that anti-torsin antibodies stain Lewy bodies and Lewy neurites in the substantia nigra and cortex. Using sensitive fluorescent resonance energy transfer (FRET) techniques, we find evidence of a close association between torsinA and α-synuclein in Lewy bodies.
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U2 - 10.1016/S0002-9440(10)61700-2
DO - 10.1016/S0002-9440(10)61700-2
M3 - Article
C2 - 11438481
AN - SCOPUS:0035404388
SN - 0002-9440
VL - 159
SP - 339
EP - 344
JO - American Journal of Pathology
JF - American Journal of Pathology
IS - 1
ER -