ITN2334
ITN2334
- Catalog: ITN2334
- Gene/Protein: TPP1 CLN2 GIG1 UNQ267/PRO304
- Product Description: Immunotag™ TPP1 Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
|
| Target Protein | TPP1 |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,ELISA |
| Recommended Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Rat,Mouse |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from human protein, at AA range: 10-90 |
| Specificity | TPP1 Polyclonal Antibody detects endogenous levels of protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. |
| Gene Name | TPP1 CLN2 GIG1 UNQ267/PRO304 |
| Accession No. | O14773 O89023 Q9EQV6 |
| Description | tripeptidyl peptidase 1(TPP1) Homo sapiens 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], |
| Cell Pathway/ Category | Lysosome, |
| Protein Expression | Adipose tissue,Brain cortex,Liver,Lymph,Placenta, |
| Subcellular Localization | mitochondrion,lysosome,melanosome,lysosomal lumen,extracellular exosome, |
| Protein 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.,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., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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