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MOCS1 Rabbit pAb

     
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
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]
Additional Information
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
StorageStore 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.

Protein Information
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
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