Rabbit Anti-MOCOS/FITC Conjugated antibody

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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.

Cat:

SL5936R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Dog,Pig,Horse,)

Immunogen：

KLH conjugated synthetic peptide derived from human MOCOS

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

98kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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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