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.
MeSH terms
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Amino Acid Sequence
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Aminopeptidases
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Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
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Endopeptidases / analysis*
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Humans
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Molecular Sequence Data
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Neuronal Ceroid-Lipofuscinoses / enzymology*
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Peptide Fragments / analysis
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Peptide Hydrolases / analysis*
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Sequence Alignment
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Serine Proteases
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Tripeptidyl-Peptidase 1
Substances
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Peptide Fragments
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Tpp1 protein, rat
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Tripeptidyl-Peptidase 1
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Endopeptidases
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Peptide Hydrolases
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Serine Proteases
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Aminopeptidases
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Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
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TPP1 protein, human