Transgenic mice with partial SUCLA2 deficiency exhibit increases in SUCLG2 expression, GTP-forming succinate-CoA ligase activity and blood carnitine estersSUCLA2 encodes the ATP-forming β subunit (A-SUCL-β) of succinyl-CoA ligase, an enzyme of the citric acid cycle. Mutations in SUCLA2 ...
These genes encode subunits of succinate-CoA ligase. Succinate-CoA ligase is a heterodimer that occurs in two forms, G-SUCL and A-SUCL. The two forms share a common a-subunit encoded by SUCLG1 and have a variant b-subunit encoded by either SUCLG2 or SUCLA2.10 G-SUCL catalyzes the ...
where V,, is the activity wrthout inhibitor. V, the activity with propionyl CoA, and n is the tiill coefficient. 1 130 STUMPF ET AL. Table 4. Acyl CoA inhibition of succinare:CoA ligasel Carnitine may prevent the adverse metabolic effects of othe ~ -- - organic acids (3. 12). ...
Transgenic mice with partial SUCLA2 deficiency exhibit increases in SUCLG2 expression, GTP-forming succinate-CoA ligase activity and blood carnitine estersdoi:10.1016/j.bbabio.2016.04.303Gergely KacsóDora RavaszJ. DocziBeáta NémethO. Madgar...
(ligase) (AMP-forming), methylmalonyl-CoA mutase, and methylmalony-CoA carboxyltransferase [71]. The genes for all of these were present in each Hermodarchaeota genome (Supplementary Table9). Subsequently, acyl-CoA produced from alkane oxidation can be oxidized to acetyl-CoA by related enzymes...