Anti -TPP1, NT (TPP1, CLN2, Tripeptidyl-peptidase 1, Cell growth-inhibiting gene 1 protein, Lysosomal pepstatin-insensitive protease, Tripeptidyl aminopeptidase, Tripeptidyl-peptidase I)

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

Chromosome: 11; NC_000011.9 (6633997..6640692, complement). Location: 11p15

Polyclonal

IgG

Rabbit

Human

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

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.

Antibodies; Abs to Enzymes, Protease (Proteinase, Peptidase)

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in Western Blot, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500

61,248 Da[Similar Products]

tripeptidyl peptidase I

tripeptidyl-peptidase 1; tripeptidyl aminopeptidase; growth-inhibiting protein 1; cell growth-inhibiting gene 1 protein; lysosomal pepstatin insensitive protease

Tripeptidyl-peptidase 1

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

TPP1_HUMAN

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

Chromosomal Location of Human Ortholog: 11p15

Cellular Component: lysosomal lumen; mitochondrion; lysosome; melanosome

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

Biological Process: nervous system development; unfolded protein response, activation of signaling protein activity; cellular protein metabolic process; epithelial cell differentiation; unfolded protein response; lysosome organization and biogenesis; peptide catabolic process; protein catabolic process; lipid metabolic process; neuromuscular process controlling balance; proteolysis; bone resorption

Disease: Ceroid Lipofuscinosis, Neuronal, 2

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