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Chemical Compound Review

propanoyl-CoA     [(2R,3S,4R,5R)-5-(6- aminopurin-9-yl)-4...

Synonyms: propionyl-CoA, Propionoyl-CoA, AG-B-45454, CHEBI:15450, CHEBI:15451, ...
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Disease relevance of Propionyl coenzyme A


High impact information on Propionyl coenzyme A

  • The diagnosis was confirmed by demonstration of deficient activity of propionyl-CoA carboxylase in cells cultured from the amniotic fluid and in fetal tissues [6].
  • We further hypothesized that replacing dietary medium-even-chain fatty acids (precursors of acetyl-CoA) by medium-odd-chain fatty acids (precursors of acetyl-CoA and anaplerotic propionyl-CoA) would restore energy production and improve cardiac and skeletal muscle function [7].
  • Here, we report the obligatory participation of HCS in the biotin-dependent stimulation of the level of HCS mRNA and those of acetyl-CoA carboxylase and the alpha subunit of propionyl-CoA carboxylase in human cells [8].
  • Expression of the cDNA clones promoted biotinylation of the bacterial biotinyl carboxyl carrier protein as well as a carboxyl-terminal fragment of the alpha subunit of human propionyl-CoA carboxylase expressed from a plasmid [9].
  • Isolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genes [10].

Chemical compound and disease context of Propionyl coenzyme A


Biological context of Propionyl coenzyme A


Anatomical context of Propionyl coenzyme A


Associations of Propionyl coenzyme A with other chemical compounds


Gene context of Propionyl coenzyme A


Analytical, diagnostic and therapeutic context of Propionyl coenzyme A


  1. Structure-based inhibitor design of AccD5, an essential acyl-CoA carboxylase carboxyltransferase domain of Mycobacterium tuberculosis. Lin, T.W., Melgar, M.M., Kurth, D., Swamidass, S.J., Purdon, J., Tseng, T., Gago, G., Baldi, P., Gramajo, H., Tsai, S.C. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  2. Mutations participating in interallelic complementation in propionic acidemia. Gravel, R.A., Akerman, B.R., Lamhonwah, A.M., Loyer, M., Léon-del-Rio, A., Italiano, I. Am. J. Hum. Genet. (1994) [Pubmed]
  3. Chaperonin-mediated assembly of wild-type and mutant subunits of human propionyl-CoA carboxylase expressed in Escherichia coli. Kelson, T.L., Ohura, T., Kraus, J.P. Hum. Mol. Genet. (1996) [Pubmed]
  4. Purification and subunit structure of propionyl coenzyme A carboxylase of Mycobacterium smegmatis. Henrikson, K.P., Allen, S.H. J. Biol. Chem. (1979) [Pubmed]
  5. Erythromycin production in Saccharopolyspora erythraea does not require a functional propionyl-CoA carboxylase. Donadio, S., Staver, M.J., Katz, L. Mol. Microbiol. (1996) [Pubmed]
  6. Prenatal diagnosis of propionic acidemia. Sweetman, L., Weyler, W., Shafai, T., Young, P.E., Nyhan, W.L. JAMA (1979) [Pubmed]
  7. Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride. Roe, C.R., Sweetman, L., Roe, D.S., David, F., Brunengraber, H. J. Clin. Invest. (2002) [Pubmed]
  8. Holocarboxylase synthetase is an obligate participant in biotin-mediated regulation of its own expression and of biotin-dependent carboxylases mRNA levels in human cells. Solórzano-Vargas, R.S., Pacheco-Alvarez, D., León-Del-Río, A. Proc. Natl. Acad. Sci. U.S.A. (2002) [Pubmed]
  9. Isolation of a cDNA encoding human holocarboxylase synthetase by functional complementation of a biotin auxotroph of Escherichia coli. León-Del-Rio, A., Leclerc, D., Akerman, B., Wakamatsu, N., Gravel, R.A. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  10. Isolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genes. Lamhonwah, A.M., Barankiewicz, T.J., Willard, H.F., Mahuran, D.J., Quan, F., Gravel, R.A. Proc. Natl. Acad. Sci. U.S.A. (1986) [Pubmed]
  11. Inhibition of hepatic propionyl-CoA synthetase activity by organic acids. Reversal of propionate inhibition of pyruvate metabolism. Krahenbuhl, S., Brass, E.P. Biochem. Pharmacol. (1991) [Pubmed]
  12. Application of a propionyl coenzyme A synthetase for poly(3-hydroxypropionate-co-3-hydroxybutyrate) accumulation in recombinant Escherichia coli. Valentin, H.E., Mitsky, T.A., Mahadeo, D.A., Tran, M., Gruys, K.J. Appl. Environ. Microbiol. (2000) [Pubmed]
  13. Genetic and biochemical characterization of the alpha and beta components of a propionyl-CoA carboxylase complex of Streptomyces coelicolor A3(2). Rodríguez, E., Gramajo, H. Microbiology (Reading, Engl.) (1999) [Pubmed]
  14. Methylmalonyl-CoA decarboxylase from Propionigenium modestum--cloning and sequencing of the structural genes and purification of the enzyme complex. Bott, M., Pfister, K., Burda, P., Kalbermatter, O., Woehlke, G., Dimroth, P. Eur. J. Biochem. (1997) [Pubmed]
  15. Acryloyl-CoA reductase from Clostridium propionicum. An enzyme complex of propionyl-CoA dehydrogenase and electron-transferring flavoprotein. Hetzel, M., Brock, M., Selmer, T., Pierik, A.J., Golding, B.T., Buckel, W. Eur. J. Biochem. (2003) [Pubmed]
  16. Coding sequence of the precursor of the beta subunit of rat propionyl-CoA carboxylase. Kraus, J.P., Firgaira, F., Novotný, J., Kalousek, F., Williams, K.R., Williamson, C., Ohura, T., Rosenberg, L.E. Proc. Natl. Acad. Sci. U.S.A. (1986) [Pubmed]
  17. Recognition, isolation, and characterization of rat liver D-methylmalonyl coenzyme A hydrolase. Kovachy, R.J., Copley, S.D., Allen, R.H. J. Biol. Chem. (1983) [Pubmed]
  18. Fatal propionic acidemia in mice lacking propionyl-CoA carboxylase and its rescue by postnatal, liver-specific supplementation via a transgene. Miyazaki, T., Ohura, T., Kobayashi, M., Shigematsu, Y., Yamaguchi, S., Suzuki, Y., Hata, I., Aoki, Y., Yang, X., Minjares, C., Haruta, I., Uto, H., Ito, Y., Müller, U. J. Biol. Chem. (2001) [Pubmed]
  19. Modification of bovine kidney pyruvate dehydrogenase kinase activity by CoA esters and their mechanism of action. Rahmatullah, M., Roche, T.E. J. Biol. Chem. (1985) [Pubmed]
  20. Sequence analysis, biogenesis, and mitochondrial import of the alpha-subunit of rat liver propionyl-CoA carboxylase. Browner, M.F., Taroni, F., Sztul, E., Rosenberg, L.E. J. Biol. Chem. (1989) [Pubmed]
  21. Analysis of genetic complementation by whole-cell microtechniques in fibroblast heterokaryons. Gravel, R.A., Leung, A., Saunders, M., Hösli, P. Proc. Natl. Acad. Sci. U.S.A. (1979) [Pubmed]
  22. Bovine kidney 3-methylcrotonyl-CoA and propionyl-CoA carboxylases: each enzyme contains nonidentical subunits. Lau, E.P., Cochran, B.C., Munson, L., Fall, R.R. Proc. Natl. Acad. Sci. U.S.A. (1979) [Pubmed]
  23. Normalization by insulin treatment of low mitochondrial glycerol phosphate dehydrogenase and pyruvate carboxylase in pancreatic islets of the GK rat. MacDonald, M.J., Efendić, S., Ostenson, C.G. Diabetes (1996) [Pubmed]
  24. A novel glucokinase regulator in pancreatic beta cells: precursor of propionyl-CoA carboxylase beta subunit interacts with glucokinase and augments its activity. Shiraishi, A., Yamada, Y., Tsuura, Y., Fijimoto, S., Tsukiyama, K., Mukai, E., Toyoda, Y., Miwa, I., Seino, Y. J. Biol. Chem. (2001) [Pubmed]
  25. Lymphocyte propionyl-CoA carboxylase and its activation by biotin are sensitive indicators of marginal biotin deficiency in humans. Stratton, S.L., Bogusiewicz, A., Mock, M.M., Mock, N.I., Wells, A.M., Mock, D.M. Am. J. Clin. Nutr. (2006) [Pubmed]
  26. A novel type of short- and medium-chain acyl-CoA hydrolases in brown adipose tissue mitochondria. Alexson, S.E., Nedergaard, J. J. Biol. Chem. (1988) [Pubmed]
  27. Metabolic effects of pent-4-enoate in isolated perfused rat heart. Hiltunen, J.K. Biochem. J. (1978) [Pubmed]
  28. Effect of hydroxycobalamin[c-lactam] on propionate and carnitine metabolism in the rat. Brass, E.P., Allen, R.H., Ruff, L.J., Stabler, S.P. Biochem. J. (1990) [Pubmed]
  29. Short-chain fatty acid synthesis in brain. Subcellular localization and changes during development. Reijnierse, G.L., Veldstra, H., Van der Ber, C.J. Biochem. J. (1975) [Pubmed]
  30. Propionic acidemia: mutation update and functional and structural effects of the variant alleles. Desviat, L.R., Pérez, B., Pérez-Cerdá, C., Rodríguez-Pombo, P., Clavero, S., Ugarte, M. Mol. Genet. Metab. (2004) [Pubmed]
  31. Down-regulated expression of PPARalpha target genes, reduced fatty acid oxidation and altered fatty acid composition in the liver of mice transgenic for hTNFalpha. Glosli, H., Gudbrandsen, O.A., Mullen, A.J., Halvorsen, B., Røst, T.H., Wergedahl, H., Prydz, H., Aukrust, P., Berge, R.K. Biochim. Biophys. Acta (2005) [Pubmed]
  32. Metabolic engineering of a novel propionate-independent pathway for the production of poly(3-hydroxybutyrate-co-3-hydroxyvalerate) in recombinant Salmonella enterica serovar typhimurium. Aldor, I.S., Kim, S.W., Prather, K.L., Keasling, J.D. Appl. Environ. Microbiol. (2002) [Pubmed]
  33. Sequence homology around the biotin-binding site of human propionyl-CoA carboxylase and pyruvate carboxylase. Lamhonwah, A.M., Quan, F., Gravel, R.A. Arch. Biochem. Biophys. (1987) [Pubmed]
  34. Low mitochondrial glycerol phosphate dehydrogenase and pyruvate carboxylase in pancreatic islets of Zucker diabetic fatty rats. MacDonald, M.J., Tang, J., Polonsky, K.S. Diabetes (1996) [Pubmed]
  35. Propionyl-coenzyme A synthase from Chloroflexus aurantiacus, a key enzyme of the 3-hydroxypropionate cycle for autotrophic CO2 fixation. Alber, B.E., Fuchs, G. J. Biol. Chem. (2002) [Pubmed]
  36. Sequence requirements for the biotinylation of carboxyl-terminal fragments of human propionyl-CoA carboxylase alpha subunit expressed in Escherichia coli. Leon-Del-Rio, A., Gravel, R.A. J. Biol. Chem. (1994) [Pubmed]
  37. Correction of the metabolic defect in propionic acidemia fibroblasts by microinjection of a full-length cDNA or RNA transcript encoding the propionyl-CoA carboxylase beta subunit. Lamhonwah, A.M., Leclerc, D., Loyer, M., Clarizio, R., Gravel, R.A. Genomics (1994) [Pubmed]
  38. Marginal maternal biotin deficiency in CD-1 mice reduces fetal mass of biotin-dependent carboxylases. Sealey, W.M., Stratton, S.L., Mock, D.M., Hansen, D.K. J. Nutr. (2005) [Pubmed]
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