Gene Symbol: Acat1
Description: acetyl-CoA acetyltransferase 1
Alias: RATACAL, acetyl-CoA acetyltransferase, mitochondrial, acetoacetyl-CoA thiolase, acetyl-Co A acetyltransferase 1 mitochondrial, acetyl-coenzyme A acetyltransferase 1
Species: rat
Products:     Acat1

Top Publications

  1. Huth W, Worm Breitgoff C, Moller U, Wunderlich I. Evidence for an in vivo modification of mitochondrial proteins by coenzyme A. Biochim Biophys Acta. 1991;1077:1-10 pubmed
    ..Thus it is evident that this coenzyme A modification is transient. We suggest that coenzyme A-modification is a signal involved in the assembly or the degradation process of distinct mitochondrial matrix proteins. ..
  2. Schwerdt G, Moller U, Huth W. Identification of the CoA-modified forms of mitochondrial acetyl-CoA acetyltransferase and of glutamate dehydrogenase as nearest-neighbour proteins. Biochem J. 1991;280 ( Pt 2):353-7 pubmed
    ..4.1.3) by amino acid sequence analysis. The results of co-immunoprecipitation and cross-linking characterize the CoA-modified forms of acetyl-CoA acetyltransferase and the glutamate dehydrogenase as nearest-neighbour proteins. ..
  3. Fukao T, Kamijo K, Osumi T, Fujiki Y, Yamaguchi S, Orii T, et al. Molecular cloning and nucleotide sequence of cDNA encoding the entire precursor of rat mitochondrial acetoacetyl-CoA thiolase. J Biochem. 1989;106:197-204 pubmed
    ..However, the purified isoenzymes were indistinguishable from each other in some analyses. Though 17 independent cDNA clones were isolated, no definite evidence indicating the presence of different cDNAs was found. ..
  4. Kim H, Vaziri N. Sterol regulatory element-binding proteins, liver X receptor, ABCA1 transporter, CD36, scavenger receptors A1 and B1 in nephrotic kidney. Am J Nephrol. 2009;29:607-14 pubmed publisher
    ..Cellular lipid homeostasis is regulated by the influx, synthesis and efflux of lipids...
  5. Forcheron F, Legedz L, Chinetti G, Feugier P, Letexier D, Bricca G, et al. Genes of cholesterol metabolism in human atheroma: overexpression of perilipin and genes promoting cholesterol storage and repression of ABCA1 expression. Arterioscler Thromb Vasc Biol. 2005;25:1711-7 pubmed
    ..It may also depend on proteins surrounding lipid droplets, adipophilin, and perilipins. They favor triglyceride storage in adipocytes and could play a similar role for cholesterol in atheroma...
  6. Morris A. Cerebral ketone body metabolism. J Inherit Metab Dis. 2005;28:109-21 pubmed
    ..The ability of KBs to act as an alternative fuel explains the effectiveness of the ketogenic diet in GLUT1 deficiency, but its effectiveness in epilepsy remains unexplained. ..
  7. Huth W, Rolle S, Wunderlich I. Turnover of matrix proteins in mammalian mitochondria. Biochem J. 2002;364:275-84 pubmed
  8. Botham K, Maldonado E, Chico Y, Zheng X, Avella M, Ochoa B. The influence of chylomicron remnants on cholesteryl ester metabolism in cultured rat hepatocytes: comparison of the effects of particles enriched in n-3 or n-6 polyunsaturated fatty acids. Biochim Biophys Acta. 2001;1534:96-109 pubmed
    ..ester hydrolases in the cytosol (cCEH) and endoplasmic reticulum (erCEH), and the expression of mRNA for ACAT1, ACAT2 and cCEH, and of enzyme protein for erCEH was determined...
  9. Bem Z, Birecka A. [A rare type of bone involvement in scleroderma (author's transl)]. Pol Przegl Radiol Med Nukl. 1978;42:115-7 pubmed

More Information


  1. Fukao T, Yamaguchi S, Scriver C, Dunbar G, Wakazono A, Kano M, et al. Molecular studies of mitochondrial acetoacetyl-coenzyme A thiolase deficiency in the two original families. Hum Mutat. 1993;2:214-20 pubmed
    ..The JB allele associates with Dutch ancestry (no consanguinity) and the JM allele with Chilean ancestry (distant consanguinity). ..
  2. Wijkhuisen A, Djouadi F, Vilar J, Merlet Benichou C, Bastin J. Thyroid hormones regulate development of energy metabolism enzymes in rat proximal convoluted tubule. Am J Physiol. 1995;268:F634-42 pubmed
    ..Altogether, these results point out the importance of the postnatal physiological rise in T3 in triggering the development of some mitochondrial oxidative enzymes in the PCT...
  3. Tian T, Ni H, Sun B. Neurobehavioral Deficits in a Rat Model of Recurrent Neonatal Seizures Are Prevented by a Ketogenic Diet and Correlate with Hippocampal Zinc/Lipid Transporter Signals. Biol Trace Elem Res. 2015;167:251-8 pubmed publisher
    ..Our findings provide support for zinc/lipid transporter signals being potential targets for the treatment of neonatal seizure-induced brain damage by KD. ..
  4. Cho K, Kim H, Kamanna V, Vaziri N. Niacin improves renal lipid metabolism and slows progression in chronic kidney disease. Biochim Biophys Acta. 2010;1800:6-15 pubmed publisher
    ..Present study sought to determine efficacy of niacin supplementation on renal tissue lipid metabolism in CRF...
  5. Haapalainen A, Meriläinen G, Pirilä P, Kondo N, Fukao T, Wierenga R. Crystallographic and kinetic studies of human mitochondrial acetoacetyl-CoA thiolase: the importance of potassium and chloride ions for its structure and function. Biochemistry. 2007;46:4305-21 pubmed
    ..The structural analysis of the active site of T2 indicates that the Phe325-Pro326 dipeptide near the catalytic cavity is responsible for the exclusive 2-methyl-branched substrate specificity. ..
  6. Kovacs W, Tape K, Shackelford J, Duan X, Kasumov T, Kelleher J, et al. Localization of the pre-squalene segment of the isoprenoid biosynthetic pathway in mammalian peroxisomes. Histochem Cell Biol. 2007;127:273-90 pubmed
  7. Kitayama K, Tanimoto T, Koga T, Terasaka N, Fujioka T, Inaba T. Importance of acyl-coenzyme A:cholesterol acyltransferase 1/2 dual inhibition for anti-atherosclerotic potency of pactimibe. Eur J Pharmacol. 2006;540:121-30 pubmed
    ..Pactimibe exhibited dual inhibition for ACAT1 and ACAT2 (concentrations inhibiting 50% [IC50s] at micromolar levels) more potently than avasimibe...
  8. Pruitt K, Maglott D. RefSeq and LocusLink: NCBI gene-centered resources. Nucleic Acids Res. 2001;29:137-40 pubmed
    ..Additional information about LocusLink and RefSeq is available at ..
  9. Fukao T, Wakazono A, Song X, Yamaguchi S, Zacharias R, Donlan M, et al. Prenatal diagnosis in a family with mitochondrial acetoacetyl-coenzyme A thiolase deficiency with the use of the polymerase chain reaction followed by the heteroduplex detection method. Prenat Diagn. 1995;15:363-7 pubmed
    ..We confirmed the diagnosis by immunoblot analysis of extracted amniocytes and gene analysis with blood filter paper after delivery. This is the first report of prenatal diagnosis of this disorder at the gene level. ..
  10. Uelmen P, Oka K, Sullivan M, Chang C, Chang T, Chan L. Tissue-specific expression and cholesterol regulation of acylcoenzyme A:cholesterol acyltransferase (ACAT) in mice. Molecular cloning of mouse ACAT cDNA, chromosomal localization, and regulation of ACAT in vivo and in vitro. J Biol Chem. 1995;270:26192-201 pubmed
    ..Two transfected Chinese hamster ovary cell lines that expressed the mouse ACAT transgene regained the ability to esterify cholesterol.(ABSTRACT TRUNCATED AT 250 WORDS) ..
  11. Escriva F, Pascual Leone A, Galan A, Encinas J. Circulating glucose, insulin and ketone bodies and enzymes of ketone body utilization in brain mitochondria from suckling rats treated with high L-thyroxine doses. Rev Esp Fisiol. 1983;39:363-71 pubmed
    ..Considering the alterations described in this paper it seems that neo-T4 syndrome could be an interesting model for studying metabolism of those substances in brain. ..
  12. Lockwood E, Bailey E. The course of ketosis and the activity of key enzymes of ketogenesis and ketone-body utilization during development of the postnatal rat. Biochem J. 1971;124:249-54 pubmed
    ..Although at all stages of development the specific activity of the heart enzyme is higher than that of brain, the total enzymic capacity of the brain is higher than that of the heart during the suckling period. ..
  13. Middleton B. The acetoacetyl-coenzyme A thiolases of rat brain and their relative activities during postnatal development. Biochem J. 1973;132:731-7 pubmed 20 days. After weaning the activity declines to 2.3 units/g fresh wt. in the adult. 5. These different developmental patterns are discussed in terms of the probable metabolic roles of the two brain acetoacetyl-CoA thiolases. ..
  14. Escriva F, Rodriguez C, Pascual Leone A. Glycemia, ketonemia, and brain enzymes of ketone body utilization in suckling and adult rats undernourished from intrauterine life. J Neurochem. 1985;44:1358-62 pubmed
    ..These results could explain the "catch up" phenomenon in several ultrastructural parameters found by other authors in undernourished postweaned rats. ..
  15. Fukao T, Yamaguchi S, Kano M, Orii T, Fujiki Y, Osumi T, et al. Molecular cloning and sequence of the complementary DNA encoding human mitochondrial acetoacetyl-coenzyme A thiolase and study of the variant enzymes in cultured fibroblasts from patients with 3-ketothiolase deficiency. J Clin Invest. 1990;86:2086-92 pubmed
    ..These results suggest that different mechanisms are involved in the enzyme defects in the four patients. ..
  16. Sokolov I, Kaigorodtseva R, Rudykh V. [Various roentgenomorphological parallels in ulcers of the pyloro-duodenal area]. Vestn Rentgenol Radiol. 1978;:3-11 pubmed
  17. Hyogo H, Roy S, Paigen B, Cohen D. Leptin promotes biliary cholesterol elimination during weight loss in ob/ob mice by regulating the enterohepatic circulation of bile salts. J Biol Chem. 2002;277:34117-24 pubmed
    ..Cholesterol gallstone formation during weight loss in ob/ob mice appears to represent a pathologic consequence of an adaptive response that prevents absorption of biliary and dietary cholesterol. ..
  18. Ozasa H, Furuta S, Miyazawa S, Osumi T, Hashimoto T, Mori M, et al. Biosynthesis of enzymes of rat-liver mitochondrial beta-oxidation. Eur J Biochem. 1984;144:453-8 pubmed
  19. Williamson D, Ilic V. Activities of enzymes of acetoacetate metabolism in rat brown adipose tissue during development. Biochem J. 1985;231:773-5 pubmed
    ..Experiments with brown-adipose-tissue slices from weanling rats indicated that 70% of the [3-14C]acetoacetate utilized was oxidized to 14CO2, and this value was not altered appreciably by the addition of glucose and insulin. ..