Reactome | MMAB adenosylates cob(I)alamin

MMAB adenosylates cob(I)alamin

Stable Identifier

R-HSA-3159253

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

2 ATP + 2 cob(II)alamin + reduced (electron-transfer flavoprotein) => 2 adenosylcob(III)alamin + 3 H(+) + oxidized (electron-transfer flavoprotein) + 2 triphosphate

ReviewStatus

5/5

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In a complex reaction, MMAB (mitochondrial cob(I)yrinic acid a,c diamide adenosyltransferase) homotrimer mediates the conversion of cob(II)alamin to a transient cob(I)alamin form which is then adenosylated to form adenosylcobalamin (AdoCbl) (Banerjee et al. 2021; Campanello et al. 2018; Fan & Bobik 2008, Leal et al. 2003; Stich et al. 2005). Defects in MMAB cause methylmalonic aciduria type cblB (MMAB aka methylmalonic aciduria type B or vitamin B12 responsive methylmalonicaciduria of cblB complementation type; MIM:251110). Affected individuals have potentially life-threatening methylmalonic aciduria and metabolic ketoacidosis, despite a functional methylmalonyl CoA mutase (Dobson et al. 2002).

Literature References

PubMed ID	Title	Journal	Year
33797888	Redox-Linked Coordination Chemistry Directs Vitamin B₁₂ Trafficking Gouda, H, Pillay, S, Banerjee, R	Acc Chem Res	2021
12438653	Identification of the gene responsible for the cblA complementation group of vitamin B12-responsive methylmalonic acidemia based on analysis of prokaryotic gene arrangements Dobson, CM, Hudson, T, Doré, C, Gravel, RA, Rosenblatt, DS, Wu, X, Leclerc, D, Wai, T, Wilson, A	Proc. Natl. Acad. Sci. U.S.A.	2002
18251506	Functional characterization and mutation analysis of human ATP:Cob(I)alamin adenosyltransferase Bobik, TA, Fan, C	Biochemistry	2008
30282455	Sacrificial Cobalt-Carbon Bond Homolysis in Coenzyme B₁₂ as a Cofactor Conservation Strategy Gouda, H, Killian, MM, Rosenblatt, DS, Gupta, A, Banerjee, R, Watkins, D, Smith, JL, Ruetz, M, Brunold, TC, Twahir, UT, Campanello, GC, Dodge, GJ, Warncke, K	J Am Chem Soc	2018
15913339	Spectroscopic evidence for the formation of a four-coordinate Co2+ cobalamin species upon binding to the human ATP:cobalamin adenosyltransferase Stich, TA, Brunold, TC, Banerjee, R, Yamanishi, M	J Am Chem Soc	2005
12514191	Identification of the human and bovine ATP:Cob(I)alamin adenosyltransferase cDNAs based on complementation of a bacterial mutant Bobik, TA, Kima, PE, Park, SD, Leal, NA	J. Biol. Chem.	2003