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Product Name MOCOS Chinese Name 原癌基因丝氨酸/苏氨酸蛋白激酶MOS抗体 Alias 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. Research Area Tumour Cell biology immunology Developmental biology Chromatin and nuclear signals Stem cells Kinases and Phosphatases Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Dog, Pig, Horse, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 98kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human MOCOS: 551-650/888 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail 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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