Homeostatic compensation maintains Ca2+ signaling functions in Purkinje neurons in the leaner mutant mouse

被引：18

作者：

Murchison D. ^{[1
]}

Dove L.S. ^{[2
]}

Abbott L.C. ^{[3
]}

Griffith W.H. ^{[1
]}

机构：

[1] Department of Medical Pharmacology and Toxicology, College of Medicine, Texas A and M University System Health Science Center, College Station

[2] PPD Development, Austin, TX

[3] Department of Veterinary Anatomy and Public Health, College of Veterinary Medicine, Texas A and M University, College Station, TX

来源：

The Cerebellum | 2002年 / 1卷 / 2期

关键词：

Ca [!sup]2+[!/sup] binding protein; Ca[!sup]2+[!/sup] buffering; Mutant mice; Voltage-gated Ca[!sup]2+[!/sup] channels;

D O I：

10.1080/147342202753671259

中图分类号：

学科分类号：

摘要：

Several human neurological disorders have been associated with mutations in the gene coding for the al subunit of the P/Q type voltage-gated calcium channel (α1A/CaV2.1). Mutations in this gene also occur in a number of neurologically afflected mouse strains, including leaner (tg1a/tg1a). Because the P-type calcium current is very prominent in cerebellar Purkinje neurons, these cells from mice with α1 subunit mutations make excellent models for the investigation of the functional consequences of native mutations in a voltage-gated calcium channel of mammalian central nervous system. In this review, we describe the impact of altered channel function on cellular calcium homeostasis and signaling. Remarkably, calcium buffering functions of the endoplasmic reticulum and calcium-binding proteins appear to be regulated in order to compensate for altered calcium influx through the mutant channels. Although this compensation may serve to maintain calcium signaling functions, such as calcium-induced calcium release, it remains uncertain whether such compensation alleviates or contributes to the behavioral phenotype. © 2002 Martin Dunitz Ltd.

引用

页码：119 / 127

页数：8

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