CA5A Antibody

產(chǎn)品詳情

Uniprot No.：

P35218
基因名：

CA5A
別名：

CA5A antibody; CA5Carbonic anhydrase 5A antibody; mitochondrial antibody; EC 4.2.1.1 antibody; Carbonate dehydratase VA antibody; Carbonic anhydrase VA antibody; CA-VA antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Synthesized peptide derived from the Internal region of Human CA VA.
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	1:40000

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.

功能：

Reversible hydration of carbon dioxide. Low activity.
基因功能參考文獻：
1. In 10 of 96 patients, mutations in CA5A were identified on both alleles but none in CA5B. Exhibiting decreased enzyme activity or thermal stability, all CAVA mutations were proven to cause disease, whereas the three variants showed no relevant effect PMID: 26913920
2. CA5A alterations cause hyperammonemia in early childhood that result in mitochondrial carbonic anhydrase VA deficiency PMID: 24530203
3. activators enhanced kcat, with no effect on KM, favoring the RDS in the catalytic cycle; the activation pattern of the two mitochondrial isoforms is very different from each other and as compared to those of the cytosolic isoforms hCA I and II. PMID: 17174092
相關(guān)疾?。?/div>
Hyperammonemia due to carbonic anhydrase VA deficiency (CA5AD)
亞細胞定位：

Mitochondrion.
蛋白家族：

Alpha-carbonic anhydrase family
數(shù)據(jù)庫鏈接：

HGNC: 1377

OMIM: 114761

KEGG: hsa:763

STRING: 9606.ENSP00000309649

UniGene: Hs.177446