Tripeptidyl Peptidase I (TPP1), Polyclonal Antibody

CLN2; GIG1; LPIC; Tripeptidyl aminopeptidase; Lysosomal pepstatin-insensitive protease; Ceroid-Lipofuscinosis, Neuronal 2, Late Infantile; Cell growth-inhibiting gene 1 protein

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

200ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-TPP1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (ELISA)

34,464 Da

tripeptidyl peptidase 1

tripeptidyl-peptidase 1

Tripeptidyl-peptidase 1

CLN2; TPP-1; LPIC; TPP-I  [Similar Products]

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. [provided by RefSeq, Jul 2008]

TPP1: 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. Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2). A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles. Belongs to the peptidase S53 family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: EC 3.4.14.9; Mitochondrial; Protease; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 11p15.4

Cellular Component: lysosomal lumen; lysosome

Molecular Function: endopeptidase activity; peptidase activity; peptide binding; protein binding; serine-type peptidase activity; tripeptidyl-peptidase activity

Biological Process: bone resorption; central nervous system development; epithelial cell differentiation; lipid metabolic process; lysosome organization and biogenesis; nervous system development; neuromuscular process controlling balance; peptide catabolic process; proteolysis

Disease: Ceroid Lipofuscinosis, Neuronal, 2

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