Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis

被引：86

作者：

Rawlings, ND ^{[1
]}

Barrett, AJ ^{[1
]}

机构：

[1] Babraham Inst, MRC, Mol Enzymol Lab, Babraham CB2 4AT, Cambs, England

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-PROTEIN STRUCTURE AND MOLECULAR ENZYMOLOGY | 1999年 / 1429卷 / 02期

关键词：

tripeptidyl-peptidase I; classical late-infantile neuronal ceroid lipofuscinosis (LINCL); CLN2; protein; pseudomonapepsin; xanthomonapepsin;

D O I：

10.1016/S0167-4838(98)00238-6

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：

We report that fragments of amino acid sequence recently described for tripeptidyl-peptidase I (TPP I)show that it is the rat homologue of the human CLN2 gene product that is deficient in classical late-infantile neuronal ceroid lipofuscinosis, This is unexpected, since the CLN2 protein has been thought to be a carboxyl-dependent endopeptidase, but TPP I is an exopeptidase possibly of serine-type. (C) 1999 Elsevier Science B.V. All rights reserved.

引用

页码：496 / 500

页数：5

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