Product Description

TPP1 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES11280-50, ES11280-100

Lead times: approximately 7-10 business days, if manufacturer has product in stock

Manufacturer/Ship Location: China

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. [provided by RefSeq, Jul 2008],

Reactivity: Human, Rat, Mouse

Host: Rabbit

Applications: WB, ELISA

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from human protein . at AA range: 10-90

Isotype: IgG

Target: TPP1,

Specificity: TPP1 Polyclonal Antibody detects endogenous levels of protein.

Dilution: WB 1:500-2000 ELISA 1:5000-20000

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formulation: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

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

Observed Band (KD): 61kD

Fields: Lysosome

Function: Catalytic Activity: Release of an N-terminal tripeptide from a polypeptide, but also has endopeptidase activity. Caution: Ref.3 sequence is wrongly reported to originate from bovine. Disease: Defects in TPP1 are the cause of classical late-infantile neuronal ceroid lipofuscinosis (LINCL) [MIM:204500]; also known as ceroid lipofuscinosis neuronal 2 (CLN2). LINCL is a fatal childhood neurodegenerative disease characterized by progressive visual and mental decline, motor disturbance, epilepsy and behavioral changes. The three main subtypes of childhood NCLs defined by the age of onset, clinical features, and ultrastructural morphology are infantile NCL (INCL), classical late-infantile NCL (LINCL), or juvenile NCL (JNCL), although a number of other distinct variants forms have been described. Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lys

Subcellular Location: Lysosome Melanosome Identified by mass spectrometry in melanosome fractions from stage I to stage IV.

Storage and Stability: -20°C/1 year

Gene Name: TPP1, CLN2, GIG1, UNQ267/PRO304

Protein Name: Tripeptidyl-peptidase 1, TPP-1, EC 3.4.14.9, Cell growth-inhibiting gene 1 protein, Lysosomal pepstatin-insensitive protease, LPIC, Tripeptidyl aminopeptidase, Tripeptidyl-peptidase I, TPP-I

Human Gene ID: 1200

Human SwissProt Number: O14773

Mouse SwissProt Number: O89023

Rat SwissProt Number: Q9EQV6

Research Use Only