[The mechanism of Lewy body formation in Parkinson's disease].
The presence of Lewy bodies(LBs) in the substantia nigra and other subcortical nuclei is a diagnostic hallmark of Parkinson's disease(PD). Recently, two mutations in the alpha-synuclein gene in families with autosomal dominant PD were identified. Subsequent immunocytochemical studies showed that antibodies to alpha-synuclein detect all of the LBs and Lewy neurites in the brains of patients with PD. Immunoelectron microscopy revealed that the reaction product is localized within abnormal filamentous structures. Moreover, alpha-synuclein is aggregated and fibrillated in vitro. More recently, a novel protein that associates with alpha-synuclein, called synphilin-1, has been reported to be present in LBs. These findings suggest that both alpha-synuclein and synphilin-1 are precise molecular compositions of LBs.
Wakabayashi
K
K
Department of Neuropathology, Hirosaki University School of Medicine.
Takahashi
H
H
D002352:Carrier Proteins / Q000378:metabolism; D006801:Humans; D016631:Lewy Bodies / Q000378:metabolism / Q000473:pathology*; D008543:Melanins / Q000378:metabolism; D009419:Nerve Tissue Proteins / Q000378:metabolism; D010300:Parkinson Disease / Q000378:metabolism / Q000473:pathology*; D013378:Substantia Nigra / Q000378:metabolism / Q000473:pathology; D051843:Synucleins; D051844:alpha-Synuclein
D002352:Carrier Proteins; D008543:Melanins; D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; C118958:SNCAIP protein, human; D051843:Synucleins; D051844:alpha-Synuclein