Recombinant Human Lysosomal protective protein (CTSA)

中文名稱：

Recombinant Human Lysosomal protective protein(CTSA)
貨號：

CSB-YP006184HU
說明書：

MSDS Datasheet
規格：
來源：

Yeast
其他：

中文名稱：

Recombinant Human Lysosomal protective protein(CTSA)
貨號：

CSB-EP006184HU
說明書：

MSDS Datasheet
規格：
來源：

E.coli
其他：

中文名稱：

Recombinant Human Lysosomal protective protein(CTSA)
貨號：

CSB-EP006184HU-B
說明書：

MSDS Datasheet
規格：
來源：

E.coli
共軛：

Avi-tag Biotinylated
E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.
其他：

中文名稱：

Recombinant Human Lysosomal protective protein(CTSA)
貨號：

CSB-BP006184HU
說明書：

MSDS Datasheet
規格：
來源：

Baculovirus
其他：

中文名稱：

Recombinant Human Lysosomal protective protein(CTSA)
貨號：

CSB-MP006184HU
說明書：

MSDS Datasheet
規格：
來源：

Mammalian cell
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

純度：

>85% (SDS-PAGE)
基因名：

CTSA
Uniprot No.：

P10619
別名：

beta galactosidase 2; BETA GALACTOSIDASE PROTECTIVE PROTEIN; beta-galactosidase 2; beta-galactosidase protective protein; betagalactosidase 2; Carboxypeptidase C; Carboxypeptidase L; carboxypeptidase Y-like kininase; Cathepsin A; Ctsa; deamidase; EC 3.4.16.5; Glactosialidosis; GLB2; Goldberg Syndrome; GSL; lysosomal carboxypeptidase A; Lysosomal protective protein 20 kDa chain; Lysosomal protective protein; Lysosomal protective protein deficiency; NEURAMINIDASE BETA GALACTOSIDASE EXPRESSION; NGBE; Neuraminidase deficiency with beta-galactosidase deficiency; NGBE; OTTHUMP00000031778; OTTHUMP00000031781 ; PPCA; PPCA deficiency; PPGB; PPGB_HUMAN; Protective protein cathepsin A; Protective protein for beta galactosidase ; Protective protein for beta-galactosidase; Protective protein/cathepsin A deficiency; urinary kininase
種屬：

Homo sapiens (Human)
蛋白長度：

Full Length of Mature Protein
表達區域：

29-480
氨基酸序列

AP DQDEIQRLPG LAKQPSFRQY SGYLKGSGSK HLHYWFVESQ KDPENSPVVL WLNGGPGCSS LDGLLTEHGP FLVQPDGVTL EYNPYSWNLI ANVLYLESPA GVGFSYSDDK FYATNDTEVA QSNFEALQDF FRLFPEYKNN KLFLTGESYA GIYIPTLAVL VMQDPSMNLQ GLAVGNGLSS YEQNDNSLVY FAYYHGLLGN RLWSSLQTHC CSQNKCNFYD NKDLECVTNL QEVARIVGNS GLNIYNLYAP CAGGVPSHFR YEKDTVVVQD LGNIFTRLPL KRMWHQALLR SGDKVRMDPP CTNTTAASTY LNNPYVRKAL NIPEQLPQWD MCNFLVNLQY RRLYRSMNSQ YLKLLSSQKY QILLYNGDVD MACNFMGDEW FVDSLNQKME VQRRPWLVKY GDSGEQIAGF VKEFSHIAFL TIKGAGHMVP TDKPLAAFTM FSRFLNKQPY
蛋白標簽：

Tag?type?will?be?determined?during?the?manufacturing?process.

The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
產品提供形式：

Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
復溶：

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
儲存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質期：

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
貨期：

Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
注意事項：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet ：

Please contact us to get it.

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
顯示更多

收起更多
相關疾病：

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