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  • Title: Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis.
    Author: Rawlings ND, Barrett AJ.
    Journal: Biochim Biophys Acta; 1999 Jan 11; 1429(2):496-500. PubMed ID: 9989235.
    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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