Primary Information
BoMiProt ID	Bomi7053
Protein Name	Methylmalonyl-CoA mutase, mitochondrial/MCM/Methylmalonyl-CoA isomerase
Organism	Bos taurus
Uniprot ID	Q9GK13
Milk Fraction	Whey
Aminoacid Length	750
Molecular Weight	83235
FASTA Sequence	Download
Gene Name	MMUT/MCM/MUT
Protein Existence Status	Reviewed
Secondary Information
Protein Function	Make succinyl-CoA which is used in Kreb's Cycle.
Biochemical Properties	Catalyzes the reversible isomerization of methylmalonyl-CoA (MMCoA) (generated from branched-chain amino acid metabolism and degradation of dietary odd chain fatty acids and cholesterol) to succinyl-CoA (3-carboxypropionyl-CoA), a key intermediate of the tricarboxylic acid cycle.Cofactor is adenosylcob(III)alamin.Inhibited by itaconyl-CoA, a metabolite that inactivates the coenzyme B12 cofactor.
Significance in milk	essential for the utilization of propionate formed during ruminal fermentation. In lactating dairy cows, propionate is the major substrate for glucose formation.
PTMs	Acetylation, Phosphorylation
Site(s) of PTM(s) N-glycosylation, O-glycosylation, Phosphorylation	>sp|Q9GK13|MUTA_BOVIN Methylmalonyl-CoA mutase, mitochondrial OS=Bos taurus OX=9913 GN=MMUT PE=2 SV=1 MLRAKNQLFLLSPHYLRQVKESSGSRLIQQRLLHQQQPLHPEWAALAKKQLKGKNPEDLI WHTPEGISIKPLYSKRDTMDLPEELPGVKPFTRGPYPTMYTFRPWTIRQYAGFSTVEESN KFYKDNIKAGQQGLSVAFDLATHRGYDSDNPRLRGDVGMAGVAIDTVEDTKILFDGIPLE KMSVSMTMNGAVIPVLATFIVTGEEQGVPKEKLTGTIQNDILKEFMVRNTYIFPPEPSMK IIADIFQYTAKHMPKFNSISISGYHMQEAGADAILELAYTIADGLEYCRTGLQAGLTIDE FAPRLSFFWGIGMNFYMEIAKMRAGRRLWAHLIEKMLQPKNSKSLLLRAHCQTSGWSLTE QDPYNNIIRTTIEAMAAVFGGTQSLHTNSFDEALGLPTVKSARIARNTQIIIQEESGIPK VADPWGGSYMMESLTNDIYDAALKLINEIEEMGGMAKAVAEGIPKLRIEECAARRQARID S*481GSEVIVGVNKYQLEKEESVDVLAIDNTSVRNKQIEKLKKVKSSRDQALAERCLDALTAC AASGDGNILALAVDATRARCTVGEITYAMKKVFGEHKANDRMVSGAYRQEFGESKEIAFA IKRVEKFMEREGRRPRLLVAKMGQDGHDRGAKVIATGFADLGFDVDIGPLFQTPREVAQQ AVDADVHTVGVSTLAAGHKTLVPELIKELNALGRPDILVMCGGVIPPQDYEFLFEVGVSN VFGPGTRIPKAAVQVLDDIEKCLEKKQQSI
Predicted Disorder Regions	NA
DisProt Annotation
TM Helix Prediction	No TM helices
Additional Comments	MUT-KO suggests the cell model to have an increased susceptibility to propionate- and H2O2-induced stress through an impairment of the mitochondrial functionality and unbalances in the oxidation-reduction processes.Methylmalonic acidemia (MMA) is a rare inborn error of metabolism caused by deficiency of the methylmalonyl-CoA mutase (MUT) enzyme.
Bibliography	1.Costanzo, M., Caterino, M., Cevenini, A., Jung, V., Chhuon, C., Lipecka, J., ... & Ruoppolo, M. (2020). Proteomics reveals that methylmalonyl-CoA mutase modulates cell architecture and increases susceptibility to stress. International journal of molecular sciences, 21(14), 4998. 2.Ruetz, M., Campanello, G. C., Purchal, M., Shen, H., McDevitt, L., Gouda, H., Wakabayashi, S., Zhu, J., Rubin, E. J., Warncke, K., Mootha, V. K., Koutmos, M., & Banerjee, R. (2019). Itaconyl-CoA forms a stable biradical in methylmalonyl-CoA mutase and derails its activity and repair. Science (New York, N.Y.), 366(6465), 589–593. https://doi.org/10.1126/science.aay0934 3.Ouattara, B., Duplessis, M., & Girard, C. L. (2013). Optimization and validation of a reversed-phase high performance liquid chromatography method for the measurement of bovine liver methylmalonyl-coenzyme a mutase activity. BMC biochemistry, 14, 25. https://doi.org/10.1186/1471-2091-14-25