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Acaa2  -  acetyl-CoA acyltransferase 2

Rattus norvegicus

Synonyms: 3-ketoacyl-CoA thiolase, mitochondrial, Acetyl-CoA acyltransferase, Beta-ketothiolase, Mitochondrial 3-oxoacyl-CoA thiolase
 
 
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Disease relevance of Acaa2

  • 3the latter two compounds are readily detoxified by the formation of N-acylglycine conjugates in liver, which may prevent large accumulations and could explain why hyperammonemia is not characteristic of patients with beta-ketothiolase deficiency or isovaleric acidemia in whom these compounds would be expected to be elevated [1].
  • Patients affected by MHBD deficiency usually manifest severe mental retardation and convulsions, whereas beta-ketothiolase-deficient patients present encephalopathic crises characterized by metabolic acidosis, vomiting and coma [2].
 

High impact information on Acaa2

 

Biological context of Acaa2

  • Messenger RNA of rat 3-oxoacyl-CoA thiolase (acetyl-CoA acyltransferase), a mitochondrial matrix enzyme involved in fatty acid beta-oxidation, was enriched by immunoprecipitation of rat liver free polysomes and recombinant plasmids were prepared from the enriched mRNA by a modification of the vector-primer method of Okayama and Berg [8].
  • Taken together, these observations indicate that aerobic energy metabolism is inhibited by MAA and to a greater extent by MHB, a fact that may be related to lactic acidaemia occurring in patients affected by MHBD and beta-ketothiolase deficiencies [2].
 

Associations of Acaa2 with chemical compounds

 

Analytical, diagnostic and therapeutic context of Acaa2

References

  1. Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia. Coude, F.X., Sweetman, L., Nyhan, W.L. J. Clin. Invest. (1979) [Pubmed]
  2. Inhibition of energy metabolism by 2-methylacetoacetate and 2-methyl-3-hydroxybutyrate in cerebral cortex of developing rats. Rosa, R.B., Schuck, P.F., de Assis, D.R., Latini, A., Dalcin, K.B., Ribeiro, C.A., da C Ferreira, G., Maria, R.C., Leipnitz, G., Perry, M.L., Filho, C.S., Wyse, A.T., Wannmacher, C.M., Wajner, M. J. Inherit. Metab. Dis. (2005) [Pubmed]
  3. cDNA-derived amino acid sequence of rat mitochondrial 3-oxoacyl-CoA thiolase with no transient presequence: structural relationship with peroxisomal isozyme. Arakawa, H., Takiguchi, M., Amaya, Y., Nagata, S., Hayashi, H., Mori, M. EMBO J. (1987) [Pubmed]
  4. Arachidonic acid formed by peroxisomal beta-oxidation of 7,10,13,16-docosatetraenoic acid is esterified into 1-acyl-sn-glycero-3-phosphocholine by microsomes. Baykousheva, S.P., Luthria, D.L., Sprecher, H. J. Biol. Chem. (1994) [Pubmed]
  5. Complete beta-oxidation of valproate: cleavage of 3-oxovalproyl-CoA by a mitochondrial 3-oxoacyl-CoA thiolase. Silva, M.F., Ruiter, J.P., Overmars, H., Bootsma, A.H., van Gennip, A.H., Jakobs, C., Duran, M., Tavares de Almeida, I., Wanders, R.J. Biochem. J. (2002) [Pubmed]
  6. Synthesis of 3-ketoacyl-CoA thiolase of rat liver peroxisomes on free polyribosomes as a larger precursor. Induction of thiolase mRNA activity by clofibrate. Fujiki, Y., Rachubinski, R.A., Mortensen, R.M., Lazarow, P.B. Biochem. J. (1985) [Pubmed]
  7. Inhibition of the glycine cleavage system by branched-chain amino acid metabolites. Kølvraa, S. Pediatr. Res. (1979) [Pubmed]
  8. Molecular cloning of cDNA for rat mitochondrial 3-oxoacyl-CoA thiolase. Miura, S., Takiguchi, M., Matsue, H., Amaya, Y., Tatibana, M., Shigesada, K., Osumi, T., Hashimoto, T., Mori, M. Eur. J. Biochem. (1986) [Pubmed]
  9. The NH2-terminal 14-16 amino acids of mitochondrial and bacterial thiolases can direct mature ornithine carbamoyltransferase into mitochondria. Arakawa, H., Amaya, Y., Mori, M. J. Biochem. (1990) [Pubmed]
 
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