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

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

Stable Identifier

R-HSA-3149563

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, mitochondrial matrix

Synonyms

MMADHC targets cytosolic cob(II)alamin for entry to mitochondria

ReviewStatus

5/5

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MMADHC targets transport of cytosolic cob(II)alamin 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 (B_12r) uptake by mitochondria (Stucki et al. 2012, Coelho et al. 2008), whereupon the complex of the 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