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TPP1 Rabbit pAb
- 产品详情
- 实验流程
Application 
| WB |
|---|---|
| Primary Accession | O14773 |
| Other Accession | O14773 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 61248 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human TPP1/CLN2 |
| Epitope Specificity | 401-500/563 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the peptidase S53 family. |
| SUBUNIT | Monomer. |
| Post-translational modifications | Activated by autocatalytic proteolytical processing upon acidification. N-glycosylation is required for processing and activity. |
| DISEASE | Involvement in disease: 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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. |
| Gene ID | 1200 |
|---|---|
| Other Names | CLN2; GIG1; LPIC; SCAR7; TPP-1; TPP1_HUMAN; TPP1; Cell growth-inhibiting gene 1 protein; Lysosomal pepstatin-insensitive protease (LPIC); Tripeptidyl aminopeptidase; Tripeptidyl-peptidase I (TPP-I); 3.4.14.9; tripeptidyl peptidase 1; ceroid-lipofuscinosis, neuronal 2, late infantile (Jansky-Bielschowsky disease); spinocerebellar ataxia, autosomal recessive 7; tripeptidyl peptidase I; TPP I; lysosomal pepstatin-insensitive carboxypeptidase |
| Dilution | WB=1:500-2000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | TPP1 |
|---|---|
| Synonyms | CLN2 |
| Function | Lysosomal serine protease with tripeptidyl-peptidase I activity (PubMed:11054422, PubMed:19038966, PubMed:19038967). May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases (PubMed:11054422, PubMed:19038966, PubMed:19038967). Requires substrates with an unsubstituted N-terminus (PubMed:19038966). |
| Cellular Location | Lysosome. Melanosome. Note=Identified by mass spectrometry in melanosome fractions from stage I to stage IV |
| Tissue Location | Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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Cat# AP97101
