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Rabbit Anti-MMACHC Antibody (CSB-PA896896ESR1HU)
Supplier: CUSABIO BIOTECH CO.
Methylmalonic aciduria and homocystinuria type C protein Antibody,MMACHC AntibodyMore alternative names for the antibody
1810037K07Rik antibody|BOS_3654 antibody|cblC antibody|DKFZp564I122 antibody|FLJ25671 antibody|Methylmalonic aciduria (cobalamin deficiency) cblC type with homocystinuria antibody|Methylmalonic aciduria and homocystinuria type C protein antibody|Methylmalonic aciduria and homocystinuria type C protein homolog antibody|MGC134307 antibody|MMAC_HUMAN antibody|MMACHC antibody|OTTHUMP00000009243 antibody|RP11 291L19.3 antibody|RP23-177C18.3 antibody
Anti-MMACHC antibody (ab187737)
Anti-MMACHC antibody (ab187737)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Methylmalonic aciduria and homocystinuria type C protein (63-282AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
Entrez Gene:513433( Cow), Entrez Gene:25974( Human), Entrez Gene:67096( Mouse), Entrez Gene:313520( Rat), Omim:609831( Human), SwissProt:Q5E9C8( Cow), SwissProt:Q9Y4U1( Human), SwissProt:Q9CZD0( Mouse), Unigene:13024( Human)
|Protein function|| |
Catalyzes the reductive dealkylation of cyanocobalamin to cob(II)alamin, using FAD or FMN as cofactor and NADPH as cosubstrate (PubMed:19700356, PubMed:21697092, PubMed:22642810). Can also catalyze the glutathione-dependent reductive demethylation of methylcobalamin, and, with much lower efficiency, the glutathione-dependent reductive demethylation of adenosylcobalamin (PubMed:19801555, PubMed:22642810, PubMed:25809485). Under anaerobic conditions cob(I)alamin is the first product; it is highly reactive and is converted to aquocob(II)alamin in the presence of oxygen (PubMed:19801555). Binds cyanocobalamin, adenosylcobalamin, methylcobalamin and other, related vitamin B12 derivatives (PubMed:21071249). .
|Protein tissue specificity|| |
Widely expressed. Expressed at higher level in fetal liver. Also expressed in spleen, lymph node, thymus and bone marrow. Weakly or not expressed in peripheral blood leukocytes. .
|Involvement in disease|| |
Methylmalonic aciduria and homocystinuria type cblC (MMAHCC) [MIM:277400]: A disorder of cobalamin metabolism characterized by decreased levels of the coenzymes adenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl). Affected individuals may have developmental, hematologic, neurologic, metabolic, ophthalmologic, and dermatologic clinical findings. Although considered a disease of infancy or childhood, some individuals develop symptoms in adulthood. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the MMACHC family.
|Protein cellular localization|| |
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|Product type|| |
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