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Cleaved-CTSA (R326) Antibody

  • 中文名稱：
    Cleaved-CTSA (R326)兔多克隆抗體
  • 貨號：
    CSB-PA000028
  • 規(guī)格：
    ￥880
  • 圖片：
    • Western Blot analysis of Jurkat cells using Cleaved-Cathepsin A 32k (R326) Polyclonal Antibody
  • 其他：

產(chǎn)品詳情

  • Uniprot No.：
  • 基因名：
  • 別名：
    beta galactosidase 2 antibody; BETA GALACTOSIDASE PROTECTIVE PROTEIN antibody; beta-galactosidase 2 antibody; beta-galactosidase protective protein antibody; betagalactosidase 2 antibody; Carboxypeptidase C antibody; Carboxypeptidase L antibody; carboxypeptidase Y-like kininase antibody; Cathepsin A antibody; Ctsa antibody; deamidase antibody; EC 3.4.16.5 antibody; Glactosialidosis antibody; GLB2 antibody; Goldberg Syndrome antibody; GSL antibody; lysosomal carboxypeptidase A antibody; Lysosomal protective protein 20 kDa chain antibody; Lysosomal protective protein antibody; Lysosomal protective protein deficiency antibody; NEURAMINIDASE BETA GALACTOSIDASE EXPRESSION; NGBE antibody; Neuraminidase deficiency with beta-galactosidase deficiency antibody; NGBE antibody; OTTHUMP00000031778 antibody; OTTHUMP00000031781 antibody; PPCA antibody; PPCA deficiency antibody; PPGB antibody; PPGB_HUMAN antibody; Protective protein cathepsin A antibody; Protective protein for beta galactosidase antibody; Protective protein for beta-galactosidase antibody; Protective protein/cathepsin A deficiency antibody; urinary kininase antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human,Mouse
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human Cathepsin A 32k.
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 抗體亞型：
    IgG
  • 純化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    WB, ELISA
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:2000
    ELISA 1:10000
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.

產(chǎn)品評價

靶點詳情

  • 功能：
    Protective protein appears to be essential for both the activity of beta-galactosidase and neuraminidase, it associates with these enzymes and exerts a protective function necessary for their stability and activity. This protein is also a carboxypeptidase and can deamidate tachykinins.
  • 基因功能參考文獻(xiàn)：
    1. The usefulness of modified U1 snRNA for rescue from exon 7 skipping caused by the IVS7 +3a>g mutation of the CTSA gene. PMID: 30010039
    2. The gene signature of OPA1, CTSA, NDUFA1, STK10 and PRDX1 was able to identify patients post-implant with a sensitivity of 91% and a specificity of 86% in discrimination between post-implant group and healthy controls. PMID: 27177495
    3. Galactosialidosis is a rare lysosomal storage disease caused by a combined deficiency of GM1 beta-galactosidase (beta-gal) and neuraminidase secondary to a defect of a lysosomal enzyme protective protein/cathepsin A (PPCA) and mutation in CTSA gene. PMID: 26259553
    4. Case Report: galactosialidosis with novel mutations of CTSA gene diagnosed using placental pathology. PMID: 25075748
    5. We identified compound heterozygous mutations in the CTSA gene, responsible for causing galactosialidosis PMID: 24769197
    6. correct nomenclature of mutations for this gene is discussed; clinical and mutational analyses of 4 cases with rare infantile form of galactosialidosis; identified 3 novel nucleotide changes, 2 resulting in missense mutations and the third, resulting in the p.Gln406* stop codon; complexity of the clinical phenotypes in GS reflects dual functions of PPCA/CTSA PMID: 23915561
    7. Catalytic function, tissue distribution and substrates of cathepsin A are discussed as well as inhibition of cathepsin A as an emerging strategy for the treatment of heart failure. PMID: 23495688
    8. The Cathepsin C releases the glycosidases from complexes formed with cathepsin A, and reinstates their activity. PMID: 22532132
    9. Our data suggest that CatA is involved in the C-terminal fine-tuning of antigenic T cell epitopes in human APC. PMID: 19954752
    10. mutations in early infantile galactosialidosis in two Dutch patients PMID: 12649068
    11. Increased activity of beta-galactosidase in the peritoneal fluid is associated with gynecologic cancers and pelvic inflammatory disease PMID: 15785934
    12. effects of GLB1, PPCA and NEU1 gene mutations on elastogenesis in skin fibroblasts PMID: 16538002
    13. Results describe the hydrodynamic properties of PPCA, NEU1, and a complex of the two proteins and identified multiple binding sites on both proteins. PMID: 19666471

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  • 相關(guān)疾?。?/div>
    Galactosialidosis (GSL)
  • 亞細(xì)胞定位：
    Lysosome.
  • 蛋白家族：
    Peptidase S10 family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 9251

    OMIM: 256540

    KEGG: hsa:5476

    STRING: 9606.ENSP00000361562

    UniGene: Hs.609336