Cell Biology Antibodies 9
Anti-TPP1 Antibody (CAB5627)
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- SKU:
- CAB5627
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
| Antibody Name: | Anti-TPP1 Antibody |
| Antibody SKU: | CAB5627 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human TPP1 (NP_000382.3). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse |
| Positive Samples: | BT-474, SW480 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human TPP1 (NP_000382.3). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | GANI STWV YSSP GRHE GQEP FLQW LMLL SNES ALPH VHTV SYGD DEDS LSSA YIQR VNTE LMKA AARG LTLL FASG DSGA GCWS VSGR HQFR PTFP ASSP YVTT VGGT SFQE PFLI TNEI VDYI SGGG FSNV FPRP SYQE EAVT KFLS SSPH LPPS SYFN ASGR AYPD VAAL SDGY WVVS NRVP IPWV SGTS ASTP VFGG ILSL INEH RILS GRPP LGFL NPRL YQQH GAGL FDVT RGCH ESCL DEEV EGQG FCSG PGWD PVTG WGTP NFPA LLKT LLNP |
| Gene ID: | 1200 |
| Uniprot: | O14773 |
| Cellular Location: | Lysosome, Melanosome |
| Calculated MW: | 34kDa/61kDa |
| Observed MW: | 50kDa |
| Synonyms: | TPP1, CLN2, GIG1, LPIC, SCAR7, TPP-1 |
| 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. |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Secreted; Protease; EC 3.4.14.9; Secreted, signal peptide; Mitochondrial 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; serine-type endopeptidase activity; metal ion binding; endopeptidase activity; peptide binding Biological Process: nervous system development; cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; 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 |
| NCBI Summary: | 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] |
| UniProt Code: | O14773 |
| NCBI GenInfo Identifier: | 108936017 |
| NCBI Gene ID: | 1200 |
| NCBI Accession: | O14773.2 |
| UniProt Secondary Accession: | O14773,Q53HT1, Q5JAK6, Q6UX56, Q71JP6, Q96C37, |
| UniProt Related Accession: | O14773 |
| Molecular Weight: | 563 |
| NCBI Full Name: | Tripeptidyl-peptidase 1 |
| NCBI Synonym Full Names: | tripeptidyl peptidase I |
| NCBI Official Symbol: | TPP1 |
| NCBI Official Synonym Symbols: | CLN2; GIG1; LPIC; SCAR7; TPP-1 |
| NCBI Protein Information: | tripeptidyl-peptidase 1; tripeptidyl aminopeptidase; growth-inhibiting protein 1; cell growth-inhibiting gene 1 protein; lysosomal pepstatin insensitive protease |
| UniProt Protein Name: | Tripeptidyl-peptidase 1 |
| UniProt Synonym Protein Names: | Cell growth-inhibiting gene 1 protein; Lysosomal pepstatin-insensitive protease; LPIC; Tripeptidyl aminopeptidase; Tripeptidyl-peptidase I; TPP-I |
| Protein Family: | Tripeptidyl-peptidase |
| UniProt Gene Name: | TPP1 |
| UniProt Entry Name: | TPP1_HUMAN |
 | Western blot analysis of extracts of various cell lines, using TPP1 antibody (CAB5627) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s. |
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