过氧化物酶体
生物
髓鞘
细胞器
肾上腺脑白质营养不良
神经科学
过氧化物酶体障碍
细胞生物学
泽尔韦格综合征
基因剔除小鼠
中枢神经系统
生物化学
受体
作者
Myriam Baes,Patrick Aubourg
标识
DOI:10.1177/1073858409336297
摘要
Peroxisomes are ubiquitous organelles with multiple metabolic functions, but their precise role in the maintenance of tissues is not well understood. All diseases caused by partial or complete peroxisome dysfunction are characterized by a variety of neurological abnormalities, underscoring the importance of peroxisomes in nervous tissue. The interrelationship between metabolic abnormalities, histological changes, and clinical signs in these peroxisomal diseases has not yet been clarified. During the past decade, a more systematic study of the consequences of peroxisome dysfunction was possible through the generation of knockout mice with generalized or conditional inactivation of peroxisomal proteins. It appears that peroxisomes are necessary for the preservation of axonal integrity and for the formation and maintenance of myelin.
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