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Comparative Study
. 1999 Jan 11;1429(2):496-500.
doi: 10.1016/s0167-4838(98)00238-6.

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

N D Rawlings  1 , A J Barrett
Affiliations
Comparative Study

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

N D Rawlings et al. Biochim Biophys Acta. .

Abstract

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.

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