Article
Adenovirus-mediated expression of mutant DRPLA proteins with expanded polyglutamine stretches in neuronally differentiated PC12 cells. Preferential intranuclear aggregate formation and apoptosis.
Human molecular genetics - 1 Jun 1999
Sato A, Shimohata T, Koide R, Takano H, Sato T, Oyake M, Igarashi S, Tanaka K, Inuzuka T, Nawa H, Tsuji S
Abstract excerpt
To investigate the molecular mechanisms of neurodegeneration caused by expanded CAG repeats in dentatorubral-pallidoluysian atrophy (DRPLA), an autosomal dominant neuro degrees enerative disorder caused by unstable expansion of a CAG trinucleotide repeat in the DRPLA gene on 12p13.31, we established an efficient expression system for truncated and full-length DRPLA proteins with normal or expanded polyglutamine...
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