Anti-MMACHC Antibody

Catalog No : IGX-43102
237.00€
175.00€

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Description 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 (PMID:18779575, PMID:19700356, PMID:21697092, PMID: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 (PMID:19801555). Forms a complex with the lysosomal transporter ABCD4 and its chaperone LMBRD1, to transport cobalamin across the lysosomal membrane into the cytosol (PMID: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 (PMID:21071249, PMID: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 (PMID:19801555, PMID:21697092, PMID:22642810, PMID: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 (PMID:19801555). Cysteine and homocysteine cannot substitute for glutathione in this reaction (PMID:19801555).
Other names Methylmalonic aciduria and homocystinuria type C protein, EC 1.16.1.-, CblC, Cyanocobalamin reductase, cyanide-eliminating,, MMACHC
Cellular localization Cytosol
Research area Signal Transduction
Note This product is for research purpose only, not for use in diagnostics or as therapeutics.
Uniprot ID Q9Y4U1
Product name Anti-MMACHC Antibody
Catalog No IGX-43102
Supplier’s Catalog No IGX-43102
Supplier ImuGeX
Host species Rabbit
Species reactivity Human
Applications ELISA, IHC
Recommended dilutions IHC: 1:20-1:200
Molecular weight N/A
Conjugate Unconjugated
Immunogen Recombinant Human Methylmalonic aciduria and homocystinuria type C protein (63-282AA)
Specificity MMACHC antibody detects endogenous level of MMACHC protein
Storage Store at +4°C for short term (1 week), for long term aliquote and store at -20°C or -80°C. Avoid repeated freeze and thaw cycles, with each freeze and thaw cycle an antibody can lose half of its binding activity.
Clonality Polyclonal
Isotype IgG
Clone ID N/A
Concentration Concentration can vary between different batches.
Storage buffer Phosphate-buffered saline (pH 7.3) containing 0.02% Sodium Azide (NaN3) and 50% Glycerol.
Formulation Liquid
Purification This antibody is immunogen affinity purified.
Handling This product contains sodium azide (NaN3) as preservative, please take appropriate care when handling this product.
Reactivity life 12 months when stored properly according to instructions (provided antibody remains sterile).