Generation and characterization of Sca2 (ataxin-2) knockout mice

被引：99

作者：

Kiehl, TR

Nechiporuk, A

Figueroa, KP

Keating, MT

Huynh, DP

Pulst, SM

机构：

[1] Cedars Sinai Med Ctr, Burns & Allen Res Inst, Rose Moss Lab Parkinson & Neurodegenerat Dis, Los Angeles, CA 90048 USA

[2] Harvard Univ, Sch Med, Childrens Hosp, Howard Hughes Med Inst, Boston, MA 02115 USA

[3] Univ Calif Los Angeles, David Geffen Sch Med, Cedars Sinai Med Ctr, Div Neurol, Los Angeles, CA 90024 USA

[4] Univ Calif Los Angeles, David Geffen Sch Med, Dept Neurobiol, Los Angeles, CA 90024 USA

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 2006年 / 339卷 / 01期

关键词：

Spinocerebellar Ataxia Type 2; SCA2; ataxin-2; A2BP1; Fox-1; knockout; obesity; rotarod; segregation distortion;

D O I：

10.1016/j.bbrc.2005.10.186

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Ataxin-2, the gene product ofthe Spinocerebellar Ataxia Type 2 (SCA2) gene, is a protein of unknown function with abundant expression in embryonic and adult tissues. Its interaction with A2BP1/Fox-1, a protein with an RNA recognition motif, suggests involvement of ataxin-2 in mRNA translation or transport. To study the effects of in vivo ataxin-2 function, we generated an ataxin-2 deficient mouse strain. Ataxin-2 deficient mice were viable. Genotypic analysis of litters from mating of heterozygous mice showed segregation distortion with a significant reduction in the birth of Sca(-/-) females. Detailed macroscopic and microscopic analysis of surviving nullizygous Sca2 knockout mice showed no major histological abnormalities. On a fat-enriched diet, ataxin-2 deficient animals had increased weight gain. Out-results demonstrate that ataxin-2, although widely expressed, is not essential in development or during adult Survival in the mouse, but leads to adult-onset obesity. (c) 2005 Elsevier Inc. All rights reserved.

引用

页码：17 / 24

页数：8

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