MMACHC Antibody (C-term) [APR08479G]

MMACHC Antibody (C-term) [APR08479G] | Gentaur UK, US & Europe Distribution

Product Category: Polyclonal Antibodies

Host: Rabbit

Species Reactivity: H

Specificity: This MMACHC antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 239-267 amino acids from the C-terminal region of human MMACHC.

Cellular Localisation: Cytoplasm, cytosol.

Molecular Weight: 31728

Clone: Polyclonal

Gene Name: MMACHC (HGNC:24525)

Gene ID: 25974

Function: Cobalamin (vitamin B12) cytosolic chaperone that catalyzes the reductive decyanation of cyanocob (III) alamin (cyanocobalamin, CNCbl) to yield cob (II) alamin and cyanide, using FAD or FMN as cofactors and NADPH as cosubstrate (PubMed:18779575, PubMed:19700356, PubMed:21697092, PubMed:25809485) . Cyanocobalamin constitutes the inactive form of vitamin B12 introduced from the diet, and is converted into the active cofactors methylcobalamin (MeCbl) involved in methionine biosynthesis, and 5'-deoxyadenosylcobalamin (AdoCbl) involved in the TCA cycle (PubMed:19801555) . Forms a complex with the lysosomal transporter ABCD4 and its chaperone LMBRD1, to transport cobalamin across the lysosomal membrane into the cytosol (PubMed:25535791) . The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR (methionine synthase reductase) and MTR (methionine synthase) which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine (PubMed:21071249, PubMed:27771510) . Also acts as a glutathione transferase by catalyzing the dealkylation of the alkylcob (III) alamins MeCbl and AdoCbl, using the thiolate of glutathione for nucleophilic displacement to generate cob (I) alamin and the corresponding glutathione thioether (PubMed:19801555, PubMed:21697092, PubMed:22642810, PubMed:25809485) . The conversion of incoming MeCbl or AdoCbl into a common intermediate cob (I) alamin is necessary to meet the cellular needs for both cofactors (PubMed:19801555) . Cysteine and homocysteine cannot substitute for glutathione in this reaction (PubMed:19801555) .

Summary: Tissue Location: 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

Form: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Application: WB

Dilution: WB--1:1000

Synonyms: Methylmalonic aciduria and homocystinuria type C protein, MMACHC