Neuronal intranuclear inclusions in spinocerebellar ataxia type 2: triple-labeling immunofluorescent study.

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 Lett

journal_title

Neuroscience letters

authors

Koyano S,Uchihara T,Fujigasaki H,Nakamura A,Yagishita S,Iwabuchi K

doi

10.1016/s0304-3940(99)00656-4

keywords:

subject

Has Abstract

pub_date

1999-10-01 00:00:00

pages

117-20

issue

2

eissn

0304-3940

issn

1872-7972

pii

S0304-3940(99)00656-4

journal_volume

273

pub_type

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