{"product_id":"abcam-ab162126","title":"Abcam, ab162126, Recombinant Human MMADHC protein","description":"\u003cp\u003eSize: 10µg\u003cbr\u003e\nRecombinant Human MMADHC protein is a Human Full Length protein, in the 1 to 296 aa range, expressed in Wheat germ, suitable for ELISA, WB.\u003cbr\u003e\nKey facts\u003cbr\u003e\nExpression system:Wheat germ,\u003cbr\u003e\nTags:GST tag N-Terminus,\u003cbr\u003e\nApplications:ELISA, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nBiologically active:No,\u003cbr\u003e\nAccession:Q9H3L0,\u003cbr\u003e\nAnimal free:No,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nSpecies:Human,\u003cbr\u003e\nStorage buffer:pH: 8Constituents: 0.79% Tris HCl, 0.31% Glutathione\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nThis product was previously labelled as C2ORF25.\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nShipped at conditions-Dry Ice, Appropriate short-term storage conditions--80°C, Appropriate long-term storage conditions--80°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze \/ thaw cycle\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nMMADHC also known as methylmalonic aciduria and homocystinuria cblD type is a protein involved in the metabolism of vitamin B12. It has a molecular weight of approximately 32 kDa. MMADHC is expressed in various tissues including liver kidney and brain. It acts as a chaperone-like factor that helps direct cobalamin into the cytosol and mitochondrian important for vitamin B12-associated reactions.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nMMADHC plays a role in the intracellular processing of vitamin B12. It is not part of a complex but interacts closely with the mitochondrial methylmalonyl-CoA mutase and cytosolic methionine synthase pathways. By assisting in cobalamin transport it enables the conversion of methylmalonic acid to succinyl-CoA and homocysteine to methionine therefore supporting essential cellular reactions.\u003cbr\u003e\nPathways\u003cbr\u003e\nMMADHC contributes to the cobalamin metabolic pathway and the methionine metabolism pathway. In the cobalamin pathway it works alongside proteins like MMACHC and MCM to facilitate vitamin B12 metabolism. It ensures that vitamin B12 reaches its target enzymes efficiently which is vital for methylation processes and energy production pathways in the mitochondria.\u003cbr\u003e\nMutations in MMADHC are linked to methylmalonic aciduria with homocystinuria cblD type. This disorder results from improper processing of vitamin B12 and often leads to neurological and metabolic complications. The protein is associated with disorders involving defective cobalamin metabolism alongside MMACHC highlighting its key role in maintaining normal vitamin B12-dependent enzyme function.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46843979563177,"sku":"ab162126","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab162126","provider":"Iright","version":"1.0","type":"link"}