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  4. Cleaved-CTSC (R394) Antibody

Cleaved-CTSC (R394) Antibody

  • 货号：
    CSB-PA000029
  • 规格：
    ￥880
  • 图片：
    • Western Blot analysis of Jurkat cells using Cleaved-Cathepsin C HC (R394) Polyclonal Antibody
  • 其他：

产品详情

  • Uniprot No.：
    P53634
  • 基因名：
  • 别名：
    AI047818 antibody; CATC antibody; CATC_HUMAN antibody; Cathepsin C antibody; Cathepsin J antibody; CPPI antibody; CTSC antibody; Dipeptidyl peptidase 1 antibody; Dipeptidyl peptidase 1 light chain antibody; Dipeptidyl peptidase I antibody; Dipeptidyl peptidase I exclusion domain chain antibody; Dipeptidyl peptidase I heavy chain antibody; Dipeptidyl peptidase I light chain antibody; Dipeptidyl transferase antibody; DPP I antibody; DPP-I antibody; DPPI antibody; EC 3.4.14.1 antibody; HMS antibody; JP antibody; JPD antibody; MGC126959 antibody; PALS antibody; PDON1 antibody; PLS antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthesized peptide derived from the C-terminal region of Human Cathepsin C HC.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    ELISA 1:20000
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    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.
  • 基因功能参考文献：
    1. expression markedly increased in the maternal vascular endothelium in subjects with preeclampsia compared with normal pregnant controls PMID: 28878298
    2. A compound mutation consisting of a large deletion and a nonsense mutation, which provides a new insight in the mutation type of CTSC gene. PMID: 26385525
    3. CTSC gene missense mutation is responsible for Papillo- Lefevre syndrome in a Turkish family. PMID: 27062382
    4. analysis of fluorescent substrates provides a detailed S' specificity study of cathepsin C PMID: 27746119
    5. Mutation screening of the CTSC gene from the two patients revealed the presence of the same homozygous nonsense mutation in Papillon-Lefevre syndrome and Haim-Munk syndromes. Phenotypic variants of the same rare disease caused by mutations of the cathepsin C (CTSC) gene. PMID: 26205983
    6. Neutrophilic Cathepsin C Is Maturated by a Multistep Proteolytic Process and Secreted by Activated Cells during Inflammatory Lung Diseases. PMID: 26884336
    7. CTSC was associated with albuminuria in type 2 diabetes patients. PMID: 26631737
    8. Homozygous mutation 901G>A in exon 7 of CTSC gene is associated with Papillon-Lefevre syndrome. PMID: 27060303
    9. Results identify a missense mutation in CTSC gene that segregate within a family with Papillon-Lefevre syndrome. PMID: 25799584
    10. Papillon-Lefevre syndrome cause by homozygous nonsense mutation of cathepsin C gene. PMID: 24894642
    11. CatC has a role in the selective tuning of innate and adaptive immune responses, relevant to a chronic immune disease, such as atherosclerosis. PMID: 25395616
    12. cathepsin C in GCF does not seem to have an effect on the pathogenesis of periodontal diseases. PMID: 24949444
    13. The present account of the 148.621 kb homozygous deletion on chromosome 11 is the first report of a mutational mechanism encompassing the whole CTSC gene. PMID: 23556547
    14. CTSC mutations in 5 Iranian families with Papillon-Lefevre syndrome (PLS)analyzed; modeled the protein for mutations found in 2 of them; presence of this mutation provides evidence for founder CTSC mutations in PLS; this P35delL mutation leads to loss of a leucine residue; results indicate the phenotypes in these 2 patients likely due to CTSC mutations PMID: 24374475
    15. Report novel deletion mutation in CTSC gene in Hungarian family with Papillon-Lefevre syndrome. PMID: 23397598
    16. Cathepsin C gene 5'-untranslated region mutation in papillon-lefevre syndrome in 4 unrelated families in Slovenia PMID: 23108224
    17. The novel loss-of function mutation of CTSC gene (c.203 T > G) found in Papillon-Lefevre Syndrome patients correlated with their diminished enzymatic activity. PMID: 23311634
    18. present a catalytic model derived from the relative rates of the acylation vs deacylation half-reactions of cathepsin C. PMID: 22928782
    19. The Cathepsin C releases the glycosidases from complexes formed with cathepsin A, and reinstates their activity. PMID: 22532132
    20. Two Indian siblings present with Haim Munk syndrome (HMS) and its cardinal features including palmoplantar keratoderma, periodontitis. arachnodactyly, acroosteolysis, onychogryphosis, osteopenia as well as allelic mutation of cathepsin C exon 6 codon. PMID: 21393975
    21. Processing of human protryptase in mast cells involves cathepsins L, B, and C. PMID: 21742978
    22. A novel mutation in the cathepsin C gene is reported in a Pakistani family with Papillon-Lefevre syndrome. PMID: 20236208
    23. This report described a novel mutation (c.267-268del)in a family with Brazilian Papillon-Lefevre syndrome and presented a review of all cathepsin C (65) mutations reported to date. PMID: 20359428
    24. study identified an identical recurrent missense mutation, R272P, in 3 families with Papillon-Lefevre syndrome(PLS); presence of this mutation in families from 2 different geographical areas provides evidence for founder effect for CTSC mutations in PLS PMID: 19816003
    25. Sequencing of the mutant cathepsin C transcript revealed that it lacked exon 3, resulting in a frameshift and introduction of a premature termination codon in Papillon-Lefevre syndrome. PMID: 11914041
    26. Selective inhibition prevents the partial processing of procaspase-3 in CD3-activated human CD8(+) T lymphocytes PMID: 12080079
    27. Identification of a novel cathepsin C mutation (p.W185X) in a Brazilian kindred with Papillon-Lefevre syndrome. PMID: 12083812
    28. All Papillon-Lefevre syndrome affected individuals from three Indian families showed three novel homozygous nonsense mutations in CTSC. PMID: 12857359
    29. Three novel CTSC missense mutations found in 21 Papillon-Lefevre syndrome families, and a complete loss of CTSC function appears to be necessary for the manifestation of this phenotype. PMID: 14974080
    30. homozygous deletion of 7 nucelotides in exon 4, creating a premature stop codon 11 amino acids downstream; 2 heterozygous missense mutations in exon 7: 1) substitution of leucine by arginine; 2)changing tryptophan to serine. PMID: 15111626
    31. This report describes the apparent reason why the study of the natural history of human patients with Papillon-Lefevre syndrome has failed to detect a generalized T cell immunodeficiency phenotype. PMID: 15585850
    32. DPP-I may play a role in converting endogenous beta-melanocortin MSH(5-22) to more potent peptides that regulate energy homeostasis in the hypothalamus. PMID: 15985311
    33. The structure of the inhibitor complex provides an explanation of the substrate specificity of hDPPI, and gives a background for the design of new inhibitors. PMID: 17020538
    34. inhibition of activation of multiple serine proteases with a cathepsin C inhibitor requires sustained exposure to prevent pro-enzyme processing PMID: 17535802
    35. Novel mutations in two Chinese patients with Papillon-Lefevre syndrome PMID: 17652201
    36. G386R missense mutation and an intragenic deletion spanning exons 3-7 and homozygous splice site mutation, p.A253SfsX30 found in papillon-Lefevre syndrome PMID: 17943190
    37. study aimed to identify CTSC mutations in different Papillon-Lefevre phenotypes, including atypical forms and isolated pre-pubertal aggressive periodontitis PMID: 18294227
    38. Cathepsin C propeptide interacts with intestinal alkaline phosphatase (IAP) and heat shock cognate protein 70. The propeptide of cathepsin C may stimulate the sorting to the lysosome contributing to the degradation of IAP in Caco-2 cells. PMID: 18307834
    39. gene variants contribute to increased susceptibility in generalized aggressive periodontitis PMID: 18809751
    40. Mutations of the cathepsin C gene are probably responsible for the phenotype of Papillon-Lefevre syndrome in this family. PMID: 18841559

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  • 相关疾病：
    Papillon-Lefevre syndrome (PLS); Haim-Munk syndrome (HMS); Periodontititis, aggressive, 1 (AP1)
  • 亚细胞定位：
    Lysosome.
  • 蛋白家族：
    Peptidase C1 family
  • 组织特异性：
    Ubiquitous. Highly expressed in lung, kidney and placenta. Detected at intermediate levels in colon, small intestine, spleen and pancreas.
  • 数据库链接：

    HGNC: 2528

    OMIM: 170650

    KEGG: hsa:1075

    STRING: 9606.ENSP00000227266

    UniGene: Hs.128065