Recombinant Human MCEE protein

Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

MCEE also known as methylmalonyl-CoA epimerase is an important enzyme with a mass of approximately 17 kDa. This enzyme is found in the mitochondria and expressed widely across different tissue types. MCEE plays a mechanical role in the conversion of D-methylmalonyl-CoA to L-methylmalonyl-CoA a necessary step in the metabolism of certain fatty acids and amino acids. The proper functioning of this enzyme is essential for efficient metabolic pathways and energy conversion in cells.

Biological function summary

Methylmalonyl-CoA epimerase contributes to the metabolism by facilitating the reversible epimerization process of methylmalonyl-CoA. MCEE is part of an enzymatic complex alongside methylmalonyl-CoA mutase which further processes the epimerized product to succinyl-CoA. This pathway is important for the breakdown and utilization of odd-chain fatty acids and certain branched-chain amino acids feeding into the citric acid cycle which is a critical pathway for cellular energy production.

Pathways

Methylmalonyl-CoA epimerase finds its place in the catabolism of β-oxidation and is directly involved in the propionic acid pathway. This positions MCEE as an integral part in facilitating the conversion of lipids and branched-chain amino acids into energy. It works closely with methylmalonyl-CoA mutase which acts downstream linking MCEE to broader metabolic processes and ensuring the continuation of energy supply within the metabolic network. This relationship is important for maintaining cellular homeostasis particularly in energy-demanding tissues.

Disruptions in the function of methylmalonyl-CoA epimerase can relate to metabolic disorders like methylmalonic acidemia. This condition arises from the accumulation of methylmalonic acid due to ineffective conversion because of enzyme defects. MCEE shows connection with proteins like methylmalonyl-CoA mutase which when dysfunctional exacerbates the metabolic imbalance. Additionally MCEE defects may influence disorders related to oxidative stress as improper metabolite handling affects energy production and cellular health.