Recombinant Human Tripeptidyl-peptidase 1 (TPP1)

Uniprot Id

O14773

Target Species

Human

Target Name

TPP1

Target Full Name

Tripeptidyl-peptidase 1

Target Function

Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus.

Target Involvement

Ceroid lipofuscinosis, neuronal, 2 (CLN2); Spinocerebellar ataxia, autosomal recessive, 7 (SCAR7)

Target Subcellular Location

Lysosome. Melanosome.

Target Tissue Specificity

Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.

Target Synonyms

Cell growth inhibiting gene 1 protein; Cell growth-inhibiting gene 1 protein; Ceroid lipofuscinosis neuronal 2; Ceroid lipofuscinosis neuronal 2 late infantile (Jansky Bielschowsky disease); Ceroid lipofuscinosis neuronal 2 late infantile; CLN 2; CLN2; GIG 1; GIG1; Growth inhibiting protein 1; LPIC; Lysosomal pepstatin insensitive protease; Lysosomal pepstatin-insensitive protease; MGC21297; TPP 1; TPP I; TPP-1; TPP-I; Tpp1; TPP1_HUMAN; TPPI; Tripeptidyl aminopeptidase; Tripeptidyl peptidase I; Tripeptidyl-peptidase 1; Tripeptidyl-peptidase I

Target Background

This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome.