Defective MMAA causes MMA, cblA type

Lerner-Ellis JP, Dobson CM, Wai T, Watkins D, Tirone JC, Leclerc D, Doré C, Lepage P, Gravel RA, Rosenblatt DS. Mutations in theMMAAgene in patients with thecblAdisorder of vitamin B12metabolism. Hum. Mutat. 2004 Nov 02;24(6):509–16. doi: 10.1002/humu.20104.; Dobson CM, Wai T, Leclerc D, Wilson A, Wu X, Doré C, Hudson T, Rosenblatt DS, Gravel RA. Identification of the gene responsible for the cblA complementation group of vitamin B 12 -responsive methylmalonic acidemia based on analysis of prokaryotic gene arrangements. Proc. Natl. Acad. Sci. U.S.A. 2002 Nov 15;99(24):15554–9. doi: 10.1073/pnas.242614799.

Metabolites



Enzyme

EC Number name full name note
5.4.99.2 methylmalonyl-CoA mutase (R)-methylmalonyl-CoA CoA-carbonylmutase


Proteins

Protein ID name full name