Mouse models for peroxisome biogenesis disorders

被引：6

作者：

Baes, M ^{[1
]}

机构：

[1] Catholic Univ Louvain, Clin Chem Lab, B-3000 Louvain, Belgium

来源：

CELL BIOCHEMISTRY AND BIOPHYSICS | 2000年 / 32卷 / 1-3期

关键词：

peroxisome; Zellweger; knockout mice; neuronal migration;

D O I：

10.1385/CBB:32:1-3:229

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：

The gene knockout technology has been applied to generate mice lacking functional peroxisomes. These mice are a model for Zellweger syndrome and other peroxisome biogenesis disorders that are lethal in early life. Extensive biochemical, ultrastructural, and neurodevelopmental analyses indicate that the peroxisome deficient mice closely mimic the pathology in Zellweger patients and will be a very useful tool to elucidate the pathogenesis of this disease.

引用

页码：229 / 237

页数：9

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