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ID 110515
タイトルヨミ
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
タイトル別表記
Cerebellar abnormalities in relation to the onset and/or development of ataxia in mice with Ca2+ channelopathy
著者
澤田, 和彦 徳島大学医学部解剖学第二講座
坂田, ひろみ 徳島大学医学部解剖学第二講座
キーワード
mutant mouse
ataxia
Ca2+ channelopathy
tyrosine hydroxylase
corticotropin-releasing factor
資料タイプ
学術雑誌論文
抄録
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.
掲載誌名
四国医学雑誌
ISSN
00373699
cat書誌ID
AN00102041
出版者
徳島医学会
57
6
開始ページ
218
終了ページ
226
並び順
218
発行日
2001-12-25
EDB ID
フルテキストファイル
言語
jpn
著者版フラグ
出版社版
部局
医学系