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MOCOS Rabbit pAb
MOCOS Rabbit pAb
- 产品详情
- 实验流程
- 背景知识
Application 
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q96EN8 |
| Predicted | Rat, Pig, Human, Mouse, Dog, Horse |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 98120 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human MOCOS |
| Epitope Specificity | 551-650/888 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 55034 |
|---|---|
| Other Names | Molybdenum cofactor sulfurase {ECO:0000255|HAMAP-Rule:MF_03050}, MCS {ECO:0000255|HAMAP-Rule:MF_03050}, MOS {ECO:0000255|HAMAP-Rule:MF_03050}, MoCo sulfurase {ECO:0000255|HAMAP-Rule:MF_03050}, hMCS, 2.8.1.9 {ECO:0000255|HAMAP-Rule:MF_03050, ECO:0000269|PubMed:34356852}, Molybdenum cofactor sulfurtransferase {ECO:0000255|HAMAP-Rule:MF_03050}, MOCOS {ECO:0000255|HAMAP-Rule:MF_03050} |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | MOCOS {ECO:0000255|HAMAP-Rule:MF_03050} |
|---|---|
| Function | Sulfurates the molybdenum cofactor (PubMed:34356852). 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 (PubMed:34356852). In vitro, the C-terminal domain is able to reduce N- hydroxylated prodrugs, such as benzamidoxime (PubMed:16973608). |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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