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 Banerjee, R, Gouda, H, Pillay, S	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, Wai, T, Leclerc, D, Wilson, A, Wu, X, Doré, C, Hudson, T, Rosenblatt, DS, Gravel, RA	Proc. Natl. Acad. Sci. U.S.A.	2002
18251506	Functional characterization and mutation analysis of human ATP:Cob(I)alamin adenosyltransferase Fan, C, Bobik, TA	Biochemistry	2008
30282455	Sacrificial Cobalt-Carbon Bond Homolysis in Coenzyme B₁₂ as a Cofactor Conservation Strategy Campanello, GC, Ruetz, M, Dodge, GJ, Gouda, H, Gupta, A, Twahir, UT, Killian, MM, Watkins, D, Rosenblatt, DS, Brunold, TC, Warncke, K, Smith, JL, Banerjee, R	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, Yamanishi, M, Banerjee, R, Brunold, TC	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 Leal, NA, Park, SD, Kima, PE, Bobik, TA	J. Biol. Chem.	2003