Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis
Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis
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DOI：
10.1016/s0167-4838(98)00238-6
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发表时间：
1999-01-11
期刊：
影响因子：
--
通讯作者：
Barrett, AJ
中科院分区：
文献类型：
--
作者：
Rawlings, ND;Barrett, AJ
我们报告，最近描述的三肽基肽酶I（TPP I）的氨基酸序列片段显示，它是人类CLN 2基因产物的大鼠同源物，在经典的婴儿晚期神经元蜡样质脂褐质沉积症中是缺乏的。这是出乎意料的，因为CLN 2蛋白被认为是一种羧基依赖性内肽酶，但TPP I是一种可能是丝氨酸型的外肽酶。(C)1999 Elsevier Science B.V.保留所有权利。
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.