Propionic acidemia (PA) and methylmalonic (MMA) are rare autosomal recessive disorders of propionyl-CoA (P-CoA) catabolism, caused by a deficiency in the enzymes P-CoA carboxylase methylmalonyl-CoA (M-CoA) mutase, respectively. PA MMA classified as intoxication-type inborn errors metabolism because intramitochondrial accumulation P-CoA, M-CoA, other metabolites results secondary inhibition mult...

Pseudomonas putida is evolutionarily endowed with features relevant for bioproduction, especially under harsh operating conditions. The rich metabolic versatility of this species, however, comes at the price limited formation acetyl-coenzyme A (CoA) from sugar substrates. Since acetyl-CoA a key precursor number added-value products, in work we deployed an silico-guided rewiring program central ...

Acetyl-CoA carboxylase catalyzes the first committed step in the synthesis of fatty acids. Because fatty acids are required during myelination in the developing brain, it was proposed that the level of acetyl-CoA carboxylase may be highest in embryonic brain. The presence of acetyl-CoA carboxylase activity was detected in chick embryo brain. Its activity varied with age, showing a peak in the 1...

The complete amino acid sequence of acetyl-CoA carboxylase from chicken liver has been deduced by cloning and sequence analysis of DNA complementary to its messenger RNA. The results were confirmed by Edman degradation of peptide fragments obtained by digestion of the enzyme polypeptide with Achromobacter proteinase I or staphylococcal serine proteinase. Chicken liver acetyl-CoA carboxylase is ...

Acetyl-CoA carboxylase phosphatase has been purified from the rat epididymal fat pad. The phosphatase occurs in a complex with the carboxylase. In the purification of the phosphatase, the high molecular weight complex was initially separated by sucrose gradient centrifugation, and the phosphatase was isolated from the complex by adjusting to 80% saturation with ethanol and by chromatography on ...

The dietary regulation of fatty acid synthesis de novo in two transplantable hepatomas, 7777 and 96188, has been studied by comparison of the acetyl coenzyme A carboxylase and fatty acid synthetase activities of tumor and host liver, both in animals fasted 48 hours and in those subsequently refed a fat-free diet for 48 hours. Neither acetyl-CoA carboxylase nor fatty acid synthetase of these hep...

Acetyl-CoA carboxylase was isolated from rat liver by polyethylene glycol precipitation and avidin affinity chromatography. Sodium dodecyl sulfate electrophoresis of the enzyme gives one protein band (Mr 250,000). Phosphate analysis of the carboxylase showed the presence of 8.3 mol of phosphate/mol of subunit (Mr 250,000). The purified carboxylase has low activity in the absence of citrate (spe...

The enzymes of fatty acid synthesis from liver and brain in normal and Blz-deprived rats were studied. Both total and specific activities of fatty acid synthetase and acetyl coenzyme A carboxylase were 2to 5-fold greater in Blz deprivation than in the normal state. The presence of excess activators in the BIp-deprived rat or an excess inhibitor in the normal rat was not found by serial admixtur...