ID 110515
Title Transcription
Ca2+チャンネルビョウ マウス ニオケル ショウノウ ノ イジョウ ト ウンドウ シッチョウ
Title Alternative
Cerebellar abnormalities in relation to the onset and/or development of ataxia in mice with Ca2+ channelopathy
Author
Sawada, Kazuhiko Department of Anatomy, The University of Tokushima School of Medicine
Sakata-Haga, Hiromi Department of Anatomy, The University of Tokushima School of Medicine
Fukui, Yoshihiro Department of Anatomy, The University of Tokushima School of Medicine Tokushima University Educator and Researcher Directory KAKEN Search Researchers
Keywords
mutant mouse
ataxia
Ca2+ channelopathy
tyrosine hydroxylase
corticotropin-releasing factor
Content Type
Journal Article
Description
This review summarizes recent studies on the morphological abnormalities of cerebella in four ataxic mutant mice, i.e., tottering mouse, leaner mouse, rolling mouse Nagoya (RMN) and rocker mouse. These mutants carry mutations in the Ca2+ channel α1A subunit gene, and become useful models for human Ca2+ channelopathy such as episodic ataxia type-2 and familial hemiplegic migraine. Abnormal expression of tyrosine hydroxylase (TH) in some Purkinje cells has been observed in tottering mice, leaner mice and RMN, but not in rocker mice. However, Purkinje cells did not seem to synthesize catecholamines. Since the transcription of the TH gene is facilitated by Ca2+, TH expression in the mutant Purkinje cells indicates functional abnormality by alterations in intracellular Ca2+ concentrations. Corticotropin-releasing factor (CRF) immunoreactivity in some climbing or mossy fibers was higher in RMN than in controls. Double immunostaining for CRF and TH revealed a correspondence in the distribution of TH-positive Purkinje cells to terminal fields of CRF-positive climbing fibers in RMN. Therefore, CRF seems to alter granule and Purkinje cell functions, such as abnormal TH expression, indicating the possible expression of ataxic symptoms.
Journal Title
四国医学雑誌
ISSN
00373699
NCID
AN00102041
Publisher
徳島医学会
Volume
57
Issue
6
Start Page
218
End Page
226
Sort Key
218
Published Date
2001-12-25
EDB ID
FullText File
language
jpn
TextVersion
Publisher
departments
Medical Sciences