MOCS1 Rabbit pAb
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q9NZB8 |
| Other Accession | Q9NZB8 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 70105 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human MOCS1 |
| Epitope Specificity | 21-120/636 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | In the C-terminal section; belongs to the moaC family. In the N-terminal section; belongs to the moaA/nifB/pqqE family. |
| Post-translational modifications | Isoform MOCS1A, isoform 2 and isoform 3 are probably thiocarboxylated at their C-terminus. Thiocarboxylation probably plays a central role in molybdenum cofactor biosynthesis, since mutagenesis of the last 2 Gly residues of isoform MOCS1A abolishes the catalytic activity of the enzyme. Thiocarboxylation is absent in isoform MOCS1B, which lacks the C-terminal Gly residue. |
| DISEASE | Defects in MOCS1 are the cause of molybdenum cofactor deficiency type A (MOCOD type A) [MIM:252150]; an autosomal recessive disease which leads to the pleiotropic loss of all molybdoenzyme activities and is characterized by severe neurological damage, neonatal seizures and early childhood death. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | Molybdenum cofactor biosynthesis is a conserved pathway leading to the biological activation of molybdenum. The protein encoded by this gene is involved in this pathway. This gene was originally thought to produce a bicistronic mRNA with the potential to produce two proteins (MOCS1A and MOCS1B) from adjacent open reading frames. However, only the first open reading frame (MOCS1A) has been found to encode a protein from the putative bicistronic mRNA, whereas additional splice variants, whose full-length natures have yet to be determined, are likely to produce a fusion between the two open reading frames. This gene is defective in patients with molybdenum cofactor deficiency, type A. A related pseudogene has been identified on chromosome 16. [provided by RefSeq, Jan 2010] |
| Gene ID | 4337 |
|---|---|
| Other Names | MIG11; MOCOD; MOCS1A; MOCS1B; 3110045D15Rik; MOCS1_BOVIN; MOCS1; MOCS1_HUMAN; Cell migration-inducing gene 11 protein; Molybdenum cofactor synthesis-step 1 protein A-B; MOCS1_MOUSE; molybdenum cofactor synthesis 1 |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=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 | MOCS1 |
|---|---|
| Function | Isoform MOCS1A and isoform MOCS1B probably form a complex that catalyzes the conversion of 5'-GTP to cyclic pyranopterin monophosphate (cPMP) (PubMed:11891227, PubMed:23627491, PubMed:29368224, PubMed:31996372). MOCS1A catalyzes the cyclization of GTP to (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate and MOCS1B catalyzes the subsequent conversion of (8S)-3',8-cyclo-7,8- dihydroguanosine 5'-triphosphate to cPMP (PubMed:11891227, PubMed:23627491, PubMed:29368224, PubMed:31996372). |
| Cellular Location | [Isoform MOCS1A]: Mitochondrion matrix [Isoform 9]: Mitochondrion matrix [Isoform 3]: Cytoplasm. Note=Found in aggregates not co- localized with mitochondria. |
| Tissue Location | Isoform MOCS1A and isoform 2 are widely expressed. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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Cat# AP99480
















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