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Chemical Compound Review

AC1L9G24     [(2R,3S,4R,5R)-5-(6-amino- 7,8-dihydropurin...

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Disease relevance of CoA

  • The model also explains underlying biochemical differences between patients with Reye syndrome and medium-chain acyl CoA dehydrogenase deficiency, another disorder of fatty acid metabolism [1].
  • Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia [2].
  • Formyl-CoA transferase catalyses transfer of CoA from formate to oxalate in the first step of oxalate degradation by Oxalobacter formigenes, a bacterium present in the intestinal flora which is implicated in oxalate catabolism in mammals [3].
  • Despite similar degrees of left ventricular systolic hypertension shortening characteristics are usually greater in patients with congenital valvular aortic stenosis (VAS) than in patients with coarctation of the aorta (CoA) [4].
  • In cultured hepatoma cells, HCV RNA replication was disrupted by treatment with lovastatin, an inhibitor of 3-hydroxy-3-methyglutaryl CoA reductase, or with an inhibitor of protein geranylgeranyl transferase I, each of which induced the dissolution of the HCV replication complex [5].

Psychiatry related information on CoA

  • Younger age at presentation, history of unexplained sibling death, a previous episode of lethargy, hypoglycemia or acidosis precipitated by fasting stress and only mildly elevated serum transaminases with normal or only mildly prolonged prothrombin time may all suggest an acyl CoA dehydrogenase deficiency [6].
  • A 31-year-old man with adult onset chorea and dementia had propionic acidemia due to propionyl CoA carboxylase deficiency [7].
  • Recent epidemiological studies suggest that inhibitors of 3-hydroxy-3-methyl-glutaryl CoA reductase, so-called statins, are effective in lowering the prevalence of Alzheimer's disease [8].
  • The risk of CoA was not associated with seasonal variation, maternal smoking, alcohol consumption, or use of deodorants [9].
  • BACKGROUND: The gene encoding fatty acid CoA ligase 4 (FACL4) is mutated in families with non-specific X linked mental retardation (MRX) and is responsible for cognitive impairment in the contiguous gene syndrome ATS-MR (Alport syndrome and mental retardation), mapped to Xq22 [10].

High impact information on CoA

  • Using an inhibitor of long-chain acyl-CoA synthetase, we demonstrate that the fatty acid has to be activated by CoA to stimulate transport [11].
  • Five cell lines (derived from three families) were able to catalyze acetylation of the lysosomal membrane and to carry out acetyl-CoA/CoA exchange, whereas a sixth cell line was devoid of this activity [12].
  • In this report, we describe the design, synthesis, and application of peptide CoA conjugates as selective HAT inhibitors for the transcriptional coactivators p300 and PCAF [13].
  • This augmenting effect of Ser-10 phosphorylation on acetylation by yGcn5 was lost by substitution of alanine for arginine 164 [Gcn5(R164A)], a residue close to Ser-10 in the structure of the ternary tGcn5/CoA/histone H3 complex [14].
  • Our screening led to the isolation of the beta subunit of human ATP-specific succinyl CoA synthetase (SCS-betaA) [2].

Chemical compound and disease context of CoA


Biological context of CoA

  • In rat hepatocytes fenofibric acid induced acyl CoA oxidase gene expression, whereas acyl CoA oxidase mRNA remained unchanged in human hepatocytes [20].
  • It is also possible that FABP, by effecting an intracellular compartmentalization of fatty acids and acyl CoA, may play a broader role in cellular lipid metabolism [21].
  • Genetic mapping of the trait placed the gene on chromosome 5 near Acads, a gene encoding the short chain acyl CoA dehydrogenase, which is mutated in BALB/cByJ mice [22].
  • Confirmation of the importance of fatty acid oxidation to thermogenesis came from our finding that mice carrying the targeted inactivation of the long chain acyl CoA dehydrogenase gene (Acadl) are also sensitive to the cold [22].
  • Formyl-CoA transferase encloses the CoA binding site at the interface of an interlocked dimer [3].

Anatomical context of CoA


Associations of CoA with other chemical compounds


Gene context of CoA

  • Our recent studies of the 455-amino acid Saccharomyces cerevisiae acyltransferase (Nmt1p) suggested that its mechanism of catalysis is ordered Bi Bi with myristoyl-CoA binding occurring prior to binding of peptide and release of CoA occurring prior to release of the myristoyl-peptide [31].
  • Also, two methods were developed to measure the binding affinities of AcCoA/CoA for GCN5 [32].
  • Human GCN5 and the N-terminal deletion mutant of PCAF are stabilized equally well by preincubation with either CoA or acetyl-CoA, while intact PCAF is better stabilized by acetyl-CoA than by CoA [33].
  • Enzyme activity measurements with expressed ACOT4 and ACOT8 show that both enzymes hydrolyze CoA esters of dicarboxylic acids with high activity but with strikingly different specificities [34].
  • When tested in a microsomal assay, Lac1p and Lag1p had a strong preference for C26:0-CoA over C24:0-CoA, C20-CoA, and C16-CoA, whereas some human homologues preferred C24:0-CoA and CoA derivatives with shorter fatty acids [35].

Analytical, diagnostic and therapeutic context of CoA

  • In-vivo and cell-culture experiments have shown that lowering the plasma cholesterol concentration or intervening in the mevalonate pathway with 3-hydroxy-3-methylglutaryl (HMG) CoA reductase inhibitors decreases tumour growth [36].
  • BACKGROUND: The study objective was to examine the vascular wall changes caused by balloon dilation of coarctation of the aorta (CoA) acutely and at short-term follow-up using intravascular ultrasound imaging [37].
  • Eleven patients with VAS (ages 6 to 41 years) and 11 with CoA were matched for age, body surface area, and peak systolic ejection gradient [4].
  • We have introduced three heterologous genes into T. vaginalis by electroporation and have used the 5' and 3' untranslated regions of the endogenous gene alpha-succinyl CoA synthetase B (alpha-SCSB) to drive transcription of these genes [38].
  • Sequence alignment of the human SCOT peptides with other known CoA transferases revealed several conserved regions of potential functional importance [39].


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  2. Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia. Furuyama, K., Sassa, S. J. Clin. Invest. (2000) [Pubmed]
  3. Formyl-CoA transferase encloses the CoA binding site at the interface of an interlocked dimer. Ricagno, S., Jonsson, S., Richards, N., Lindqvist, Y. EMBO J. (2003) [Pubmed]
  4. Altered left ventricular mechanics in patients with valvular aortic stenosis and coarction of the aorta: effects on systolic performance and late outcome. Borow, K.M., Colan, S.D., Neumann, A. Circulation (1985) [Pubmed]
  5. Disruption of hepatitis C virus RNA replication through inhibition of host protein geranylgeranylation. Ye, J., Wang, C., Sumpter, R., Brown, M.S., Goldstein, J.L., Gale, M. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  6. Medium-chain and long-chain acyl CoA dehydrogenase deficiency: clinical, pathologic and ultrastructural differentiation from Reye's syndrome. Treem, W.R., Witzleben, C.A., Piccoli, D.A., Stanley, C.A., Hale, D.E., Coates, P.M., Watkins, J.B. Hepatology (1986) [Pubmed]
  7. Adult-onset chorea and dementia with propionic acidemia. Sethi, K.D., Ray, R., Roesel, R.A., Carter, A.L., Gallagher, B.B., Loring, D.W., Hommes, F.A. Neurology (1989) [Pubmed]
  8. High doses of simvastatin, pravastatin, and cholesterol reduce brain cholesterol synthesis in guinea pigs. Lütjohann, D., Stroick, M., Bertsch, T., Kühl, S., Lindenthal, B., Thelen, K., Andersson, U., Björkhem, I., Bergmann Kv, K., Fassbender, K. Steroids (2004) [Pubmed]
  9. Risk factors for coarctation of the aorta. Tikkanen, J., Heinonen, O.P. Teratology (1993) [Pubmed]
  10. A third MRX family (MRX68) is the result of mutation in the long chain fatty acid-CoA ligase 4 (FACL4) gene: proposal of a rapid enzymatic assay for screening mentally retarded patients. Longo, I., Frints, S.G., Fryns, J.P., Meloni, I., Pescucci, C., Ariani, F., Borghgraef, M., Raynaud, M., Marynen, P., Schwartz, C., Renieri, A., Froyen, G. J. Med. Genet. (2003) [Pubmed]
  11. Fatty acyl-coenzyme A is required for budding of transport vesicles from Golgi cisternae. Pfanner, N., Orci, L., Glick, B.S., Amherdt, M., Arden, S.R., Malhotra, V., Rothman, J.E. Cell (1989) [Pubmed]
  12. Genetic evidence for transmembrane acetylation by lysosomes. Bame, K.J., Rome, L.H. Science (1986) [Pubmed]
  13. HATs off: selective synthetic inhibitors of the histone acetyltransferases p300 and PCAF. Lau, O.D., Kundu, T.K., Soccio, R.E., Ait-Si-Ali, S., Khalil, E.M., Vassilev, A., Wolffe, A.P., Nakatani, Y., Roeder, R.G., Cole, P.A. Mol. Cell (2000) [Pubmed]
  14. Phosphorylation of serine 10 in histone H3 is functionally linked in vitro and in vivo to Gcn5-mediated acetylation at lysine 14. Lo, W.S., Trievel, R.C., Rojas, J.R., Duggan, L., Hsu, J.Y., Allis, C.D., Marmorstein, R., Berger, S.L. Mol. Cell (2000) [Pubmed]
  15. Acetyl CoA carboxylase in cultured fibroblasts: differential biotin dependence in the two types of biotin-responsive multiple carboxylase deficiency. Packman, S., Caswell, N., Gonzalez-Rios, M.C., Kadlecek, T., Cann, H., Rassin, D., McKay, C. Am. J. Hum. Genet. (1984) [Pubmed]
  16. The nonredundant roles of two 4'-phosphopantetheinyl transferases in vital processes of Mycobacteria. Chalut, C., Botella, L., de Sousa-D'Auria, C., Houssin, C., Guilhot, C. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  17. Inhibition of hydroxymethylglutaryl coenzyme A reductase activity induces a paradoxical increase in DNA synthesis in myeloid leukemia cells. Hohl, R.J., Larson, R.A., Mannickarottu, V., Yachnin, S. Blood (1991) [Pubmed]
  18. Acetoacetate coenzyme A transferase activity in rat hepatomas. Fenselau, A., Wallis, K., Morris, H.P. Cancer Res. (1975) [Pubmed]
  19. Intestinal and hepatic cholesterogenesis in hypercholesterolemic dyslipidemia of experimental diabetes in dogs. Kwong, L.K., Feingold, K.R., Peric-Golia, L., Le, T., Karkas, J.D., Alberts, A.W., Wilson, D.E. Diabetes (1991) [Pubmed]
  20. Fibrates downregulate apolipoprotein C-III expression independent of induction of peroxisomal acyl coenzyme A oxidase. A potential mechanism for the hypolipidemic action of fibrates. Staels, B., Vu-Dac, N., Kosykh, V.A., Saladin, R., Fruchart, J.C., Dallongeville, J., Auwerx, J. J. Clin. Invest. (1995) [Pubmed]
  21. Fatty acid binding protein. Role in esterification of absorbed long chain fatty acid in rat intestine. Ockner, R.K., Manning, J.A. J. Clin. Invest. (1976) [Pubmed]
  22. Abnormal nonshivering thermogenesis in mice with inherited defects of fatty acid oxidation. Guerra, C., Koza, R.A., Walsh, K., Kurtz, D.M., Wood, P.A., Kozak, L.P. J. Clin. Invest. (1998) [Pubmed]
  23. Regulation of rat liver hydroxymethylglutaryl coenzyme A reductase by a new class of noncompetitive inhibitors. Effects of dichloroacetate and related carboxylic acids on enzyme activity. Stacpoole, P.W., Harwood, H.J., Varnado, C.E. J. Clin. Invest. (1983) [Pubmed]
  24. Relationship between the coenzyme A and the carnitine pools in human skeletal muscle at rest and after exhaustive exercise under normoxic and acutely hypoxic conditions. Friolet, R., Hoppeler, H., Krähenbühl, S. J. Clin. Invest. (1994) [Pubmed]
  25. Coenzyme A sequestration in rat hearts oxidizing ketone bodies. Russell, R.R., Taegtmeyer, H. J. Clin. Invest. (1992) [Pubmed]
  26. Biotin-responsive alopecia and developmental regression. Charles, B.M., Hosking, G., Green, A., Pollitt, R., Bartlett, K., Taitz, L.S. Lancet (1979) [Pubmed]
  27. Partial deletion of membrane-bound domain of 3-hydroxy-3-methylglutaryl coenzyme A reductase eliminates sterol-enhanced degradation and prevents formation of crystalloid endoplasmic reticulum. Jingami, H., Brown, M.S., Goldstein, J.L., Anderson, R.G., Luskey, K.L. J. Cell Biol. (1987) [Pubmed]
  28. Activation of cholesterol synthesis in preference to fatty acid synthesis in liver and adipose tissue of transgenic mice overproducing sterol regulatory element-binding protein-2. Horton, J.D., Shimomura, I., Brown, M.S., Hammer, R.E., Goldstein, J.L., Shimano, H. J. Clin. Invest. (1998) [Pubmed]
  29. Oxidation of pristanic acid in fibroblasts and its application to the diagnosis of peroxisomal beta-oxidation defects. Paton, B.C., Sharp, P.C., Crane, D.I., Poulos, A. J. Clin. Invest. (1996) [Pubmed]
  30. Feedback regulation of bile-acid synthesis in the rat. Differing effects of taurocholate and tauroursocholate. Shefer, S., Nguyen, L., Salen, G., Batta, A.K., Brooker, D., Zaki, F.G., Rani, I., Tint, G.S. J. Clin. Invest. (1990) [Pubmed]
  31. Use of photoactivatable peptide substrates of Saccharomyces cerevisiae myristoyl-CoA:protein N-myristoyltransferase (Nmt1p) to characterize a myristoyl-CoA-Nmt1p-peptide ternary complex and to provide evidence for an ordered reaction mechanism. Rudnick, D.A., Rocque, W.J., McWherter, C.A., Toth, M.V., Jackson-Machelski, E., Gordon, J.I. Proc. Natl. Acad. Sci. U.S.A. (1993) [Pubmed]
  32. Kinetic mechanism of the histone acetyltransferase GCN5 from yeast. Tanner, K.G., Langer, M.R., Kim, Y., Denu, J.M. J. Biol. Chem. (2000) [Pubmed]
  33. The histone acetyltransferase activity of human GCN5 and PCAF is stabilized by coenzymes. Herrera, J.E., Bergel, M., Yang, X.J., Nakatani, Y., Bustin, M. J. Biol. Chem. (1997) [Pubmed]
  34. The identification of a succinyl-CoA thioesterase suggests a novel pathway for succinate production in peroxisomes. Westin, M.A., Hunt, M.C., Alexson, S.E. J. Biol. Chem. (2005) [Pubmed]
  35. Human homologues of LAG1 reconstitute Acyl-CoA-dependent ceramide synthesis in yeast. Guillas, I., Jiang, J.C., Vionnet, C., Roubaty, C., Uldry, D., Chuard, R., Wang, J., Jazwinski, S.M., Conzelmann, A. J. Biol. Chem. (2003) [Pubmed]
  36. Cholesterol inhibition, cancer, and chemotherapy. Buchwald, H. Lancet (1992) [Pubmed]
  37. Acute and follow-up intravascular ultrasound findings after balloon dilation of coarctation of the aorta. Sohn, S., Rothman, A., Shiota, T., Luk, G., Tong, A., Swensson, R.E., Sahn, D.J. Circulation (1994) [Pubmed]
  38. Transient and selectable transformation of the parasitic protist Trichomonas vaginalis. Delgadillo, M.G., Liston, D.R., Niazi, K., Johnson, P.J. Proc. Natl. Acad. Sci. U.S.A. (1997) [Pubmed]
  39. Succinyl CoA: 3-oxoacid CoA transferase (SCOT): human cDNA cloning, human chromosomal mapping to 5p13, and mutation detection in a SCOT-deficient patient. Kassovska-Bratinova, S., Fukao, T., Song, X.Q., Duncan, A.M., Chen, H.S., Robert, M.F., Pérez-Cerdá, C., Ugarte, M., Chartrand, C., Vobecky, S., Kondo, N., Mitchell, G.A. Am. J. Hum. Genet. (1996) [Pubmed]
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