Human B12-dependent enzymes: Methionine synthase and Methylmalonyl-CoA mutase. [PDF]
Methods Enzymol, 2022 Humans have only two known cobalamin or B12-dependent enzymes: cytoplasmic methionine synthase and mitochondrial methylmalonyl-CoA mutase. A complex intracellular B12 trafficking pathway, comprising a multitude of chaperones, process and deliver cobalamin to the two target enzymes.
Mascarenhas R +3 more
europepmc +4 more sources
Label-Free Quantitative Proteomics in a Methylmalonyl-CoA Mutase-Silenced Neuroblastoma Cell Line [PDF]
International Journal of Molecular Sciences, 2018 Methylmalonic acidemias (MMAs) are inborn errors of metabolism due to the deficient activity of methylmalonyl-CoA mutase (MUT). MUT catalyzes the formation of succinyl-CoA from methylmalonyl-CoA, produced from propionyl-CoA catabolism and derived from ...
Marianna Caterino +2 more
exaly +5 more sources
Chemistry Journal of Moldova: General, Industrial and Ecological Chemistry, 2023 The in vivo experiments show that the adenosylcobalamin cofactor in glutamate mutase and methylmalonyl-CoA mutase processes lose its dimethylbenzimidazole axial ligand before starting the enzymatic processes.
Tudor Spataru
doaj +2 more sources
Adenoviral-mediated correction of methylmalonyl-CoA mutase deficiency in murine fibroblasts and human hepatocytes [PDF]
BMC Medical Genetics, 2007 Background Methylmalonic acidemia (MMA), a common organic aciduria, is caused by deficiency of the mitochondrial localized, 5'deoxyadenosylcobalamin dependent enzyme, methylmalonyl-CoA mutase (MUT).
Korson Mark +7 more
doaj +3 more sources
Studies on Methylmalonyl-CoA Mutase from Escherichia coli [PDF]
, 2008 Methylmalonyl-CoA mutase (MCM, E.C. 5.4.99.2), a coenzyme B12-dependent enzyme, catalyses the inter conversion of succinyl-CoA and methylmalonyl-CoA. The gene (sbm) encoding this enzyme is found in Escherichia coli (E. coli) at 62.3min on the E. coli chromosome. However, the metabolic role of this enzyme in the organism is not known.
Kannan, S.M.
openaire +2 more sources
Cofactor Selectivity in Methylmalonyl Coenzyme A Mutase, a Model Cobamide-Dependent Enzyme [PDF]
mBio, 2019 Cobamides, a uniquely diverse family of enzyme cofactors related to vitamin B12, are produced exclusively by bacteria and archaea but used in all domains of life.
Olga M. Sokolovskaya +5 more
doaj +2 more sources
Understanding the off-loading mechanism of adenosylcobalamin by Cupriviadus metallidurans adenosyltransferase from C. metallidurans Isobutyryl-CoA Mutase Fused [PDF]
Structural DynamicsEnzymes are Nature's highly efficient catalysts, driving the metabolism of diverse substrates essential for sustaining life across all biological kingdoms.
Jayoh Amurao Hernandez
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Naturally occurring cobalamin (B12) analogs can function as cofactors for human methylmalonyl-CoA mutase. [PDF]
Biochimie, 2021 Cobalamin, commonly known as vitamin B12, is an essential micronutrient for humans because of its role as an enzyme cofactor. Cobalamin is one of over a dozen structurally related compounds - cobamides - that are found in certain foods and are produced ...
Sokolovskaya OM +7 more
europepmc +2 more sources
Proteomics Reveals That Methylmalonyl-CoA Mutase Modulates Cell Architecture and Increases Susceptibility to Stress [PDF]
International Journal of Molecular Sciences, 2020 Marianna Caterino +2 more
exaly +2 more sources
Intragenic complementation in methylmalonyl CoA mutase
, 1994 Methylmalonic aciduria (MMA) is an autosomal recessive metabolic disorder with an incidence of 1 in 48,000, which may be due to a defect in the mitochondrial homodimeric enzyme methylmalonyl CoA mutase (mut MMA). mut MMA is subdivided into $mut sp circ$ and $mut sp-$ subclasses on the basis of complementation analysis; $mut sp circ$ cell lines have ...
Farah, Rita S.
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