MMADHC targets transport of cytosolic cob(II)alamin to mitochondria

Stable Identifier
Reaction [uncertain]
Homo sapiens
MMADHC targets cytosolic cob(II)alamin for entry to mitochondria
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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).

Literature References
PubMed ID Title Journal Year
22156578 Molecular mechanisms leading to three different phenotypes in the cblD defect of intracellular cobalamin metabolism

Coelho, D, Baumgartner, MR, Suormala, T, Burda, P, Stucki, M, Fowler, B

Hum. Mol. Genet. 2012
18385497 Gene identification for the cblD defect of vitamin B12 metabolism

Coelho, D, Baumgartner, MR, Rosenblatt, DS, Suormala, T, Newbold, RF, Stucki, M, Fowler, B, Lerner-Ellis, JP

N. Engl. J. Med. 2008
33797888 Redox-Linked Coordination Chemistry Directs Vitamin B12 Trafficking

Gouda, H, Pillay, S, Banerjee, R

Acc Chem Res 2021
Orthologous Events
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