Glutamate Dehydrogenase in Cerebellar Mutant Mice: Gene Localization and Enzyme Activity in Different Tissues
作者:
Odile Miret‐Duvaux,
Florence Frederic,
Dominique Simon,
Jean‐Louis Guenet,
André Hanauer,
Nicole Delhaye‐Bouchaud,
Jean Mariani,
期刊:
Journal of Neurochemistry
(WILEY Available online 1990)
卷期:
Volume 54,
issue 1
页码: 23-29
ISSN:0022-3042
年代: 1990
DOI:10.1111/j.1471-4159.1990.tb13278.x
出版商: Blackwell Publishing Ltd
关键词: Olivopontocerebellar atrophies;Multiple system atrophies;Neurological mutant mouse;Glutamate dehydrogenase;Gene localization
数据来源: WILEY
摘要:
Abstract:Many similarities of both the inheritance pattern and the neuropathology can be observed between olivopontocerebellar atrophies, or so‐called multiple system atrophies (MSAs), and murine cerebellar mutations like Purkinje cell degeneration, nervous, staggerer, weaver, and reeler. Our study aimed to test whether the glutamate dehydrogenase (GDH) deficiency observed in some MSA patients could be found also in any of the murine mutants. GDH activity was assayed in several organs of these mutants, and no general deficiency was detected. By contrast, the level was found to be elevated in the cerebellum. The GDH gene was localized on mouse chromosome 14 and does not map close to any known neurological mutation in the mouse. We conclude, for the moment, that none of these cerebellar mutant mice can be considered as an animal model for GDH‐deficient
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