Anti-CTSC Antibody (CAB8403)
- SKU:
- CAB8403
- Product type:
- Antibody
- Application:
- WB
- Application:
- IHC
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-CTSC Antibody |
抗体コード: | CAB8403 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-134 of human CTSC (NP_001805.3). |
申し込み: | WB IHC |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:100 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | NCI-H460, 293T, SW620, HL-60, HeLa, Mouse liver |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 25-134 of human CTSC (NP_001805.3). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | DTPA NCTY LDLL GTWV FQVG SSGS QRDV NCSV MGPQ EKKV VVYL QKLD TAYD DLGN SGHF TIIY NQGF EIVL NDYK WFAF FKYK EEGS KVTT YCNE TMTG WVHD VLGR NW |
遺伝子ID: | 1075 |
Uniprot: | P53634 |
セルラーロケーション: | Lysosome |
計算された分子量: | 15kDa/51kDa |
観察された分子量: | 70kDa |
同義語: | CTSC, CPPI, DPP-I, DPP1, DPPI, HMS, JP, JPD, PALS, PDON1, PLS |
バックグラウンド: | This gene encodes a member of the peptidase C1 family and lysosomal cysteine proteinase that appears to be a central coordinator for activation of many serine proteinases in cells of the immune system. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate heavy and light chains that form a disulfide-linked dimer. A portion of the propeptide acts as an intramolecular chaperone for the folding and stabilization of the mature enzyme. This enzyme requires chloride ions for activity and can degrade glucagon. Defects in the encoded protein have been shown to be a cause of Papillon-Lefevre syndrome, an autosomal recessive disorder characterized by palmoplantar keratosis and periodontitis. |
UniProt Protein Function: | CTSC: Thiol protease. Has dipeptidylpeptidase activity. Active against a broad range of dipeptide substrates composed of both polar and hydrophobic amino acids. Proline cannot occupy the P1 position and arginine cannot occupy the P2 position of the substrate. Can act as both an exopeptidase and endopeptidase. Activates serine proteases such as elastase, cathepsin G and granzymes A and B. Can also activate neuraminidase and factor XIII. Defects in CTSC are a cause of Papillon-Lefevre syndrome (PLS); also known as keratosis palmoplantaris with periodontopathia. PLS is an autosomal recessive disorder characterized by palmoplantar keratosis and severe periodontitis affecting deciduous and permanent dentitions and resulting in premature tooth loss. The palmoplantar keratotic phenotype vary from mild psoriasiform scaly skin to overt hyperkeratosis. Keratosis also affects other sites such as elbows and knees. Defects in CTSC are a cause of Haim-Munk syndrome (HMS); also known as keratosis palmoplantaris with periodontopathia and onychogryposis or Cochin Jewish disorder. HMS is an autosomal recessive disorder characterized by palmoplantar keratosis, onychogryphosis and periodontitis. Additional features are pes planus, arachnodactyly, and acroosteolysis. Defects in CTSC are a cause of aggressive periodontititis type 1 (AP1); also known as juvenile periodontitis (JPD) and prepubertal periodontitis (PPP). AP1 is characterized by severe and protracted gingival infections, leading to tooth loss. AP1 inheritance is autosomal dominant. Belongs to the peptidase C1 family. 3 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:EC 3.4.14.1; Endoplasmic reticulum; Protease Chromosomal Location of Human Ortholog: 11q14.2 Cellular Component: Golgi apparatus; extracellular space; membrane; lysosome; endoplasmic reticulum Molecular Function:identical protein binding; protein binding; protein self-association; chaperone binding; serine-type endopeptidase activity; apoptotic protease activator activity; chloride ion binding; cysteine-type peptidase activity; phosphatase binding Biological Process: response to organic substance; apoptosis; immune response; proteolysis; T cell mediated cytotoxicity; aging Disease: Periodontitis, Aggressive, 1; Papillon-lefevre Syndrome; Haim-munk Syndrome |
NCBI Summary: | The protein encoded by this gene, a member of the peptidase C1 family, is a lysosomal cysteine proteinase that appears to be a central coordinator for activation of many serine proteinases in immune/inflammatory cells. It is composed of a dimer of disulfide-linked heavy and light chains, both produced from a single protein precursor, and a residual portion of the propeptide acts as an intramolecular chaperone for the folding and stabilization of the mature enzyme. This enzyme requires chloride ions for activity and can degrade glucagon. Defects in the encoded protein have been shown to be a cause of Papillon-Lefevre syndrome, an autosomal recessive disorder characterized by palmoplantar keratosis and periodontitis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | P53634 |
NCBI GenInfo Identifier: | 88683003 |
NCBI Gene ID: | 1075 |
NCBI Accession: | AAI13851.1 |
UniProt Secondary Accession: | P53634,Q2HIY8, Q53G93, Q71E75, Q71E76, Q7M4N9, Q7Z3G7 Q7Z5U7, Q8WY99, Q8WYA7, A8K7V2, B5MDD5, |
UniProt Related Accession: | P53634 |
Molecular Weight: | 51,854 Da |
NCBI Full Name: | CTSC protein |
NCBI Synonym Full Names: | cathepsin C |
NCBI Official Symbol: | CTSC |
NCBI Official Synonym Symbols: | JP; HMS; JPD; PLS; CPPI; DPP1; DPPI; PALS; DPP-I; PDON1 |
NCBI Protein Information: | dipeptidyl peptidase 1; cathepsin J; dipeptidyl peptidase I; dipeptidyl transferase; dipeptidyl-peptidase I |
UniProt Protein Name: | Dipeptidyl peptidase 1 |
UniProt Synonym Protein Names: | Cathepsin C; Cathepsin J; Dipeptidyl peptidase I; DPP-I; DPPI; Dipeptidyl transferaseCleaved into the following 3 chains:Dipeptidyl peptidase 1 exclusion domain chain; Alternative name(s):; Dipeptidyl peptidase I exclusion domain chainDipeptidyl peptidase 1 heavy chain; Alternative name(s):; Dipeptidyl peptidase I heavy chainDipeptidyl peptidase 1 light chain; Alternative name(s):; Dipeptidyl peptidase I light chain |
Protein Family: | Cathepsin |
UniProt Gene Name: | CTSC |
UniProt Entry Name: | CATC_HUMAN |
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