A5010083558- Porphyrin Metabolism and Disorders
- Metabolism and Genetic Disorders
- Trace Elements in Health
- Iron Metabolism and Disorders
- Folate and B Vitamins Research
- Hemoglobinopathies and Related Disorders
- Biochemical and Molecular Research
- Enzyme Structure and Function
Massachusetts Institute of Technology
2022-2023
G-protein metallochaperone MeaB in bacteria [methylmalonic aciduria type A (MMAA) humans] is responsible for facilitating the delivery of adenosylcobalamin (AdoCbl) to methylmalonyl-CoA mutase (MCM), only AdoCbl-dependent enzyme humans. Genetic defects switch III region MMAA lead genetic disorder methylmalonic which body unable process certain lipids. Here, we present a crystal structure Methylobacterium extorquens bound nonhydrolyzable guanosine triphosphate (GTP) analog...
G-protein metallochaperones are essential for the proper maturation of numerous metalloenzymes. The chaperone MMAA in humans (MeaB bacteria) uses GTP hydrolysis to facilitate delivery adenosylcobalamin (AdoCbl) AdoCbl-dependent methylmalonyl-CoA mutase, an metabolic enzyme. This also facilitates removal damaged cobalamin (Cbl) repair. Although most chaperones standalone proteins, isobutyryl-CoA mutase fused (IcmF) has a domain covalently attached its target mutase. We previously showed that...