Abstract:
:Spinocerebellar ataxia type 2 (SCA2) is associated with an expansion of CAG/polyglutamine-repeat of a gene of unknown function. We performed an immunohistochemical study to identify the immunolocalization of the disease protein ataxin-2 in normal and SCA2 patients. Although normal and expanded ataxin-2 were ubiquitously localized to the cytoplasm of neurons, ubiquitinated intranuclear inclusions were observed selectively in 1-2% of neurons of affected brain regions except the cerebellum. Triple-labeling immunofluorescence revealed that ataxin-2, expanded polyglutamine and ubiquitin were colocalized to these neuronal intranuclear inclusions (NIs), indicating that SCA2 shares morphological characteristics common to other neurological disorders associated with an expansion of CAG/polyglutamine-repeat. Lack of NIs in the cerebellar lesion, however, suggests the discrepancy between formation of NIs and neuronal degeneration in SCA2.
journal_name
Neurosci Lettjournal_title
Neuroscience lettersauthors
Koyano S,Uchihara T,Fujigasaki H,Nakamura A,Yagishita S,Iwabuchi Kdoi
10.1016/s0304-3940(99)00656-4keywords:
subject
Has Abstractpub_date
1999-10-01 00:00:00pages
117-20issue
2eissn
0304-3940issn
1872-7972pii
S0304-3940(99)00656-4journal_volume
273pub_type
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