Proteins Involved in the Intracellular Metabolism of Foes ait Gene proposed Functions (Abbreviation) Name ATP-binding cassette | Chl] _] This ATP-binding cassette transporter is involved in the export of subfamily D vitamin B,> from the lysosome; this function may occur as the result member 4 (ABCD4) of direct, active export activity or indirectly via interactions with LMBD1. LMBDI Domain CbIF_ | This lipocalin receptor-like transmembrane protein is involved in the Containing 1 export of vitamin By) from the lysosome, either through passive (LMBD1) transfer or interactions with ABCD4. Methylmalonic CbIC_ | This protein immediately binds “base off” vitamin B,2 following export aciduria and from the lysosome, with broad specificity for upper axial ligands; it homocystinuria functions to heterolytically and homolytically cleave cobalt-carbon. type C protein bonds, removing the upper axial ligand of vitamin Bp. (MMACHC) Methylmalonic CbID_ | This protein appears not to bind vitamin B,3 disectly but rather functions aciduria and as an adaptor protein for MMACHC, assisting in its trafficking and homocystinuria binding to acceptor proteins typeD protein (MMADHC) Methylmalonic CIA | MMAA isa P-loop GTPase that serves as a G protein chaperone and aciduria type A functions to remove inactive cob(I)alamin from the active site of (MMAA) methylmalonyl-CoA mutase. ATP-dependent biB- | ATR is a bifunctional mitochondrial enzyme that catalyzes the transfer cob(alamin of an adenosyl group to cob(1)alamin and coordinates adenosyitransferase adenosylcobalamin transfer to methylmalonyl-CoA mutase; itmay (ATR) facilitate coupling to flavoprotein oxidoreductases to reduce cob(I)alamin to cob({)alamin, enabling the transfer of the adenosyl group. Methionine synthase | CbIG | MTR catalyzes the two-step transfer of a methyl group from (ir) methyltetrahydrofolate to homocysteine, A methyl group is first transferred from methylCbl to generate homocysteine, followed by the removal of the methyl group from methyltetrahydrofolate by cob(I)alamin. Methionine synthase | CbIE | Cob()alamin generated in the methionine synthase reaction can reductase (MTRR) undergo oxidation to the inactive cob(Malamin. MTER is a diflavin oxidoreductase that uses NADPH and S-adenosylmethionine to reduce the oxidized cob(I)alamin in the methionine synthase reaction. This represents a mechanism for generating methylcobalamin for methionine synthase reactions in situ, ATP, Adenosine triphosphate; ATR, ATP-dependent cob(I)alamin adenosyltransferase; CoA, coenzyme A; GTP, guanosine 5'-triphosphate; NADPH, reduced nicotinamide adenine dinucleotide phosphate.