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Rabbit Anti-MOCOS/RBITC Conjugated antibody (bs-5936R-RBITC)
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-5936R-RBITC
英文名稱 Rabbit Anti-MOCOS/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標(biāo)記的原癌基因絲氨酸/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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  發(fā)育生物學(xué)  染色質(zhì)和核信號(hào)  干細(xì)胞  激酶和磷酸酶  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Horse, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 98kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MOCOS
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 background:
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.

Database links:

Entrez Gene: 55034 Human

Omim: 613274 Human

SwissProt: Q96EN8 Human



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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