BD-PN2334

TPP1 Rabbit Polyclonal Antibody

常规抗体

TPP1 CLN2 GIG1 UNQ267/PRO304

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)

WB

Human,Rat,Mouse

1 mg/ml

Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.

1200

O14773

https://www.uniprot.org/uniprotkb/O14773/entry

O89023

http://www.uniprot.org/uniprot/O89023

Q9EQV6

http://www.uniprot.org/uniprot/O54941Q9EQV6

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

TPP1 Polyclonal Antibody detects endogenous levels of protein.

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

61kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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],

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

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

Lysosome;

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.,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.,online information:Neural Ceroid Lipofuscinoses mutation db,PTM:Activated by autocatalytic proteolytical processing upon acidification.,similarity:Belongs to the peptidase S53 family.,subcellular location:Identified by mass spectrometry in melanosome fractions from stage I to stage IV.,tissue specificity:Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.,

现货

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