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Rabbit Anti-MOCS1/Cy7 Conjugated antibody (bs-17700R-Cy7)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-17700R-Cy7
英文名称 Anti-MOCS1/Cy7
中文名称 Cy7标记的钼辅因子合成蛋白1抗体
别    名 Cell migration-inducing gene 11 protein; MIG11; MOCOD; Mocs1; MOCS1_HUMAN; Molybdenum cofactor biosynthesis protein 1; Molybdenum cofactor biosynthesis protein A; Molybdenum cofactor biosynthesis protein C; Molybdenum cofactor synthesis 1; Molybdenum cofactor synthesis-step 1 protein A-B.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  神经生物学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human, Mouse, Rat, Pig, Horse, Rabbit, )
产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 70kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MOCS1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
Molybdenum cofactor biosynthesis is a conserved pathway leading to the biological activation of molybdenum. The protein encoded by this gene is involved in this pathway. This gene was originally thought to produce a bicistronic mRNA with the potential to produce two proteins (MOCS1A and MOCS1B) from adjacent open reading frames. However, only the first open reading frame (MOCS1A) has been found to encode a protein from the putative bicistronic mRNA, whereas additional splice variants, whose full-length natures have yet to be determined, are likely to produce a fusion between the two open reading frames. This gene is defective in patients with molybdenum cofactor deficiency, type A. A related pseudogene has been identified on chromosome 16. [provided by RefSeq, Jan 2010]

Function:
Isoform MOCS1A and isoform MOCS1B probably form a complex that catalyzes the conversion of a guanosine derivative to precursor Z during molybdenum cofactor biosynthesis.

Tissue Specificity:
Isoform MOCS1A and isoform 2 are widely expressed.

Post-translational modifications:
Isoform MOCS1A, isoform 2 and isoform 3 are probably thiocarboxylated at their C-terminus. Thiocarboxylation probably plays a central role in molybdenum cofactor biosynthesis, since mutagenesis of the last 2 Gly residues of isoform MOCS1A abolishes the catalytic activity of the enzyme. Thiocarboxylation is absent in isoform MOCS1B, which lacks the C-terminal Gly residue.

DISEASE:
Defects in MOCS1 are the cause of molybdenum cofactor deficiency type A (MOCOD type A) [MIM:252150]; an autosomal recessive disease which leads to the pleiotropic loss of all molybdoenzyme activities and is characterized by severe neurological damage, neonatal seizures and early childhood death.

Similarity:
In the C-terminal section; belongs to the moaC family.
In the N-terminal section; belongs to the moaA/nifB/pqqE family.

Database links:

Entrez Gene: 4337 Human

Entrez Gene: 56738 Mouse

Entrez Gene: 301221 Rat

Omim: 603707 Human

SwissProt: Q1JQD7 Cow

SwissProt: Q9NZB8 Human

SwissProt: Q5RKZ7 Mouse

Unigene: 718492 Human

Unigene: 22256 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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