Methylmalonyl-CoA mutase catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA, and uses a B12 derived prosthetic group, adenosylcobalamin, in order to accomplish this transfer.
32.
PurE, " N " 5-carboxyaminoimidazole ribonucleotide mutase, converts N5-CAIR to CAIR, the sixth step of " de novo " purine biosynthesis.
33.
L-" threo "-3-Methylaspartate "'is an unusual amino acid formed by glutamate mutase and can be metabolised by methylaspartate ammonia-lyase.
34.
Phosphoglycerate mutase B ( PGM-B ) is one of the latter glycolytic enzymes responsible for the conversion of 3-phosphoglycerate ( 3PG ) to 2-phosphoglycerate ( 2PG ).
35.
The inherited forms of methylmalonic acidemia cause defects in the metabolic pathway where methylmalonyl-coenzyme A ( CoA ) is converted into succinyl-CoA by the enzyme methylmalonyl-CoA mutase.
36.
Patients with a complete deletion suffer an inactivation of methylmalonyl CoA mutase and exhibit the most sever symptoms of the deficiency, while patients with a partial mutations have a wide range of symptoms.
37.
Because phosphoenolpyruvate mutase has the unusual ability to form a new carbon-phosphorus bond, it is essential to the synthesis of phosphonates, such as phosphonolipids and the antibiotics fosfomycin and bialaphos.
38.
Dysfunction in the activity of phosphoglycerate mutase is a rare autosomal recessive genetic disorder with symptoms ranging from mild to moderate, is not thought life-threatening and can be managed with changes in lifestyle.
39.
The coenzyme A linked form of methylmalonic acid, methylmalonyl-CoA, is converted into succinyl-CoA by methylmalonyl-CoA mutase, in a reaction that requires vitamin B 12 as a cofactor.
40.
All these propionyl-CoA substrates are converted to succinyl-CoA following the methylmalonyl pathway For amino acid metabolism, methylmalonyl-CoA mutase works in the degradation pathways of isoleucine, threonine, valine, and methionine.