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迟发性神经脂质沉积症的生化基础。

Biochemical basis of late-onset neurolipidoses.

作者信息

Conzelmann E, Sandhoff K

机构信息

Physiologisch-Chemisches Institut, Universität Würzburg, BRD.

出版信息

Dev Neurosci. 1991;13(4-5):197-204. doi: 10.1159/000112160.

DOI:10.1159/000112160
PMID:1817024
Abstract

The occurrence and the tremendous phenotypic variability of late-onset neurolipidosis variants are explained on the basis of a simple kinetic model that describes the correlation between residual activity of a deficient lysosomal enzyme and the degradation rate of its substrate in the lysosome.

摘要

迟发性神经脂质沉积症变体的发生及其巨大的表型变异性是基于一个简单的动力学模型来解释的，该模型描述了缺陷溶酶体酶的残余活性与其在溶酶体中底物降解速率之间的相关性。

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