Cleaved-CTSA (R326) Antibody

說明書

中文名稱：

Cleaved-CTSA (R326)兔多克隆抗體
貨號：

CSB-PA000028
規格：

￥1090
圖片：
Western Blot analysis of Jurkat cells using Cleaved-Cathepsin A 32k (R326) Polyclonal Antibody
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

P10619
基因名：

CTSA
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.
產品提供形式：

Liquid
應用范圍：

WB, ELISA

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:2000
ELISA	1:10000

Protocols：

Western Blotting (WB) Protocol
ELISA Protocol
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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

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：
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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相關疾?。?/div>
Galactosialidosis (GSL)
亞細胞定位：

Lysosome.
蛋白家族：

Peptidase S10 family
數據庫鏈接：

HGNC: 9251

OMIM: 256540

KEGG: hsa:5476

STRING: 9606.ENSP00000361562

UniGene: Hs.609336

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