Reaction: MMADHC targets transport of cytosolic cob(II)alamin to mitochondria
- in pathway: Cobalamin (Cbl) metabolism
MMADHC (Methylmalonic aciduria and homocystinuria type D protein) has sequence homology with a bacterial ATP binding cassette transporter and contains a putative cobalamin binding motif and a putative mitochondrial targeting sequence. It is required for cob(II)alamin (B12r) uptake by mitochondria (Stucki et al. 2012, Coelho et al. 2008), whereupon the complex ofthe two proteins dissociates, but the actual mechanism of transport into the mitochondrion is unknown (Banerjee et al. 2021).
Reaction - small molecule participants:
cob(II)alamin [mitochondrial matrix]
Reactome.org reaction link: R-HSA-3149563
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Reaction input - small molecules:
Reaction output - small molecules:
cob(II)alamin
Reactome.org link: R-HSA-3149563