MOCS2 Antibody, FITC conjugated

产品详情

产品名称：

Rabbit anti-Homo sapiens (Human) MOCS2 Polyclonal antibody
Uniprot No.：

O96007
基因名：

MOCS2
别名：

MOCS2 antibody; MCBPE antibody; MOCO1 antibody; Molybdopterin synthase catalytic subunit antibody; EC 2.8.1.12 antibody; MOCO1-B antibody; Molybdenum cofactor synthesis protein 2 large subunit antibody; Molybdenum cofactor synthesis protein 2B antibody; MOCS2B antibody; Molybdopterin-synthase large subunit antibody; MPT synthase large subunit antibody
宿主：

Rabbit
反应种属：

Human
免疫原：

Recombinant Human Molybdopterin synthase catalytic subunit protein (1-188AA)
免疫原种属：

Homo sapiens (Human)
标记方式：

FITC
克隆类型：

Polyclonal
抗体亚型：

IgG
纯化方式：

>95%, Protein G purified
浓度：

It differs from different batches. Please contact us to confirm it.
保存缓冲液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
产品提供形式：

Liquid
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.
用途：

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

功能：

Catalytic subunit of the molybdopterin synthase complex, a complex that catalyzes the conversion of precursor Z into molybdopterin. Acts by mediating the incorporation of 2 sulfur atoms from thiocarboxylated MOCS2A into precursor Z to generate a dithiolene group.
基因功能参考文献：
1. analysis of sites within MOCS2 where mutations cause molybdenum cofactor deficiency PMID: 12732628
2. There was undisturbed production of both transcripts, while Western blot analysis demonstrated that MOCS2B, the large subunit, is unstable in the absence of MOCS2A. PMID: 16737835
3. We reported here an infant with MoCD who presented with hypoxic ischaemic encephalopathy and identified a novel mutation, c.130C>T in cDNA of the MOCS2 gene from the infant. PMID: 17158010
相关疾病：

Molybdenum cofactor deficiency, complementation group B (MOCODB)
亚细胞定位：

Cytoplasm, cytosol.
蛋白家族：

MoaE family, MOCS2B subfamily
组织特异性：

Highest levels are found in heart and skeletal muscle. Lower levels are present in brain, kidney and pancreas. Very low levels are found in lung and peripheral blood leukocytes.
数据库链接：

HGNC: 7193

OMIM: 252160

KEGG: hsa:4338

STRING: 9606.ENSP00000380157

UniGene: Hs.163645