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Rabbit Anti-MOCOS  antibody (bs-5936R)  
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50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
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產(chǎn)品編號 bs-5936R
英文名稱 Rabbit Anti-MOCOS  antibody
中文名稱 原癌基因絲氨酸/蘇氨酸蛋白激酶MOS抗體
別    名 c mos; c-mos; MSV; y Oocyte maturation factor mos; Proto oncogene serine / threonine protein kinase mos; FLJ20733; HMCS; MCS; MoCo sulfurase; Molybdenum cofactor sulfurase; MOS; v mos Moloney murine sarcoma viral oncogene homolog; MOCOS_HUMAN.  
研究領域 腫瘤  細胞生物  免疫學  發(fā)育生物學  染色質和核信號  干細胞  激酶和磷酸酶  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human,Mouse,Rat,Pig,Dog,Horse)
產(chǎn)品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,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 98kDa
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MOCOS: 551-650/888 
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(chǎn)品介紹 MOCOS sulfurates the molybdenum cofactor of xanthine dehydrogenase (XDH; MIM 607633) and aldehyde oxidase (AOX1; MIM 602841), which is required for their enzymatic activities (Ichida et al., 2001 [PubMed 11302742]).[supplied by OMIM, Feb 2010]

Function:
Sulfurates the molybdenum cofactor. Sulfation of molybdenum is essential for xanthine dehydrogenase (XDH) and aldehyde oxidase (ADO) enzymes in which molybdenum cofactor is liganded by 1 oxygen and 1 sulfur atom in active form.

DISEASE:
Defects in MOCOS are the cause of xanthinuria type 2 (XU2) [MIM:603592]. Xanthinuria is characterized by excretion of very large amounts of xanthine in the urine and a tendency to form xanthine stones. Uric acid is strikingly diminished in serum and urine. In addition, patient suffering of XU2 cannot metabolize allopurinol into oxypurinol due to dual deficiency of xanthine dehydrogenase and aldehyde oxidase.

Similarity:
Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family.
MOCOS subfamily.
Contains 1 MOSC domain.

SWISS:
Q96EN8

Gene ID:
55034

Database links:

Entrez Gene: 55034 Human

Omim: 613274 Human

SwissProt: Q96EN8 Human



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