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        MOCS1 Rabbit pAb (bs-17700R)  
        訂購熱線:400-901-9800
        訂購郵箱:sales@bioss.com.cn
        訂購QQ:  400-901-9800
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        50ul/1180.00元
        100ul/1980.00元
        200ul/2800.00元
        大包裝/詢價
        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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