Methylmalonyl-CoA - Wikipedia
Methylmalonyl-CoA is the thioester consisting of coenzyme A linked to methylmalonic acid. It is an important intermediate in the biosynthesis of succinyl-CoA , which plays an essential role in …
Guidelines for the diagnosis and management of methylmalonic acidaemia ...
This first revision of the MMA and PA guidelines covers the same subtypes of isolated MMA and PA as before. MMA is caused by a deficiency of the methylmalonyl‐CoA mutase enzyme …
Methylmalonyl-CoA | C25H40N7O19P3S | CID 123909 - PubChem
Methylmalonyl-CoA | C25H40N7O19P3S | CID 123909 - structure, chemical names, physical and chemical properties, classification, patents, literature, biological activities, …
Human B12-dependent enzymes: Methionine synthase and Methylmalonyl-CoA …
Humans have only two known cobalamin or B12-dependent enzymes: cytoplasmic methionine synthase and mitochondrial methylmalonyl-CoA mutase. A complex intracellular B12 …
Methylmalonyl-CoA - an overview | ScienceDirect Topics
May 4, 1999 · Propionyl CoA is converted to D-methylmalonyl CoA in a reaction catalyzed by the biotin-dependent propionyl CoA carboxylase. Propionyl CoA and methylmalonyl CoA can also …
New insights into the pathophysiology of methylmalonic acidemia
Beyond acylation, numerous functions have been attributed to the reversible interaction of MMA metabolites such as methylmalonyl-CoA, methylmalonic acid, and propionate with enzyme …
Isolated Methylmalonic Acidemia - GeneReviews® - NCBI Bookshelf
Aug 16, 2005 · For this GeneReview, the term "isolated methylmalonic acidemia" refers to a group of inborn errors of metabolism associated with elevated methylmalonic acid (MMA) …
Methylmalonyl-CoA - an overview | ScienceDirect Topics
In methylmalonic acidemia (MMA), the conversion of methylmalonyl-CoA to succinyl-CoA is impaired secondary to the reduced activity of methylmalonyl-CoA mutase, a vitamin B12 …
Methylmalonyl-CoA - AMBOSS
Formed from propionyl-CoA by propionyl-CoA carboxylase and converted by methylmalonyl-CoA mutase to succinyl-CoA, which then enters the TCA cycle. © 2024 AMBOSS | Terms and …
Integrated multi-omics reveals anaplerotic rewiring in methylmalonyl …
Jan 26, 2023 · Our results highlight the importance of the loss of methylmalonyl-CoA as an anaplerotic source and indicate a relevant reduction of TCA cycle intermediates in MMA.