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MOCS1 Rabbit pAb (bs-17700R)  
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50ul/1180.00元
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產品編號 bs-17700R
英文名稱 MOCS1 Rabbit pAb
中文名稱 鉬輔因子合成蛋白1抗體
別    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.  
研究領域 細胞生物  神經生物學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 70 kDa
檢測分子量
細胞定位 細胞核 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MOCS1: 21-120/636 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
Q9NZB8

Gene ID:
4337

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



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