PubMed

Carnitine insufficiency caused by aging and overnutrition compromises mitochondrial performance and metabolic control.

Noland RC, Koves TR, Seiler SE, Lum H, Lust RM, Ilkayeva O, Stevens RD, Hegardt FG, Muoio DM.

J Biol Chem. 2009 Aug 21;284(34):22840-52. Epub 2009 Jun 24.PMID: 19553674 [PubMed - indexed for MEDLINE]Related articles

Novel role of FATP1 in mitochondrial fatty acid oxidation in skeletal muscle cells.

Sebastián D, Guitart M, García-Martínez C, Mauvezin C, Orellana-Gavaldà JM, Serra D, Gómez-Foix AM, Hegardt FG, Asins G.

J Lipid Res. 2009 Sep;50(9):1789-99. Epub 2009 May 9.PMID: 19429947 [PubMed - in process]Related articlesFree article

Ten novel HMGCL mutations in 24 patients of different origin with 3-hydroxy-3-methyl-glutaric aciduria.

Menao S, López-Viñas E, Mir C, Puisac B, Gratacós E, Arnedo M, Carrasco P, Moreno S, Ramos M, Gil MC, Pié A, Ribes A, Pérez-Cerda C, Ugarte M, Clayton PT, Korman SH, Serra D, Asins G, Ramos FJ, Gómez-Puertas P, Hegardt FG, Casals N, Pié J.

Hum Mutat. 2009 Mar;30(3):E520-9.PMID: 19177531 [PubMed - indexed for MEDLINE]Related articles

C75 is converted to C75-CoA in the hypothalamus, where it inhibits carnitine palmitoyltransferase 1 and decreases food intake and body weight.

Mera P, Bentebibel A, López-Viñas E, Cordente AG, Gurunathan C, Sebastián D, Vázquez I, Herrero L, Ariza X, Gómez-Puertas P, Asins G, Serra D, García J, Hegardt FG.

Biochem Pharmacol. 2009 Mar 15;77(6):1084-95. Epub 2008 Nov 27.PMID: 19094968 [PubMed - indexed for MEDLINE]Related articles

CPT1c is localized in endoplasmic reticulum of neurons and has carnitine palmitoyltransferase activity.

Sierra AY, Gratacós E, Carrasco P, Clotet J, Ureña J, Serra D, Asins G, Hegardt FG, Casals N.

J Biol Chem. 2008 Mar 14;283(11):6878-85. Epub 2008 Jan 11. Erratum in: J Biol Chem. 2008 May 2;283(18):12680. PMID: 18192268 [PubMed - indexed for MEDLINE]Related articlesFree article

A new LC-ESI-MS/MS method to measure long-chain acylcarnitine levels in cultured cells.

Jáuregui O, Sierra AY, Carrasco P, Gratacós E, Hegardt FG, Casals N.

Anal Chim Acta. 2007 Sep 5;599(1):1-6. Epub 2007 Aug 3.PMID: 17765057 [PubMed - indexed for MEDLINE]Related articles

Molecular genetics of HMG-CoA lyase deficiency.

Pié J, López-Viñas E, Puisac B, Menao S, Pié A, Casale C, Ramos FJ, Hegardt FG, Gómez-Puertas P, Casals N.

Mol Genet Metab. 2007 Nov;92(3):198-209. Epub 2007 Aug 9. Review.PMID: 17692550 [PubMed - indexed for MEDLINE]Related articles

C-terminal end and aminoacid Lys48 in HMG-CoA lyase are involved in substrate binding and enzyme activity.

Carrasco P, Menao S, López-Viñas E, Santpere G, Clotet J, Sierra AY, Gratacós E, Puisac B, Gómez-Puertas P, Hegardt FG, Pie J, Casals N.

Mol Genet Metab. 2007 Jun;91(2):120-7. Epub 2007 Apr 24.PMID: 17459752 [PubMed - indexed for MEDLINE]Related articles

Definition by functional and structural analysis of two malonyl-CoA sites in carnitine palmitoyltransferase 1A.

López-Viñas E, Bentebibel A, Gurunathan C, Morillas M, de Arriaga D, Serra D, Asins G, Hegardt FG, Gómez-Puertas P.

J Biol Chem. 2007 Jun 22;282(25):18212-24. Epub 2007 Apr 23.PMID: 17452323 [PubMed - indexed for MEDLINE]Related articlesFree article

Modulating aroma compounds during wine fermentation by manipulating carnitine acetyltransferases in Saccharomyces cerevisiae.

Cordente AG, Swiegers JH, Hegardt FG, Pretorius IS.

FEMS Microbiol Lett. 2007 Feb;267(2):159-66. Epub 2006 Nov 29.PMID: 17156120 [PubMed - indexed for MEDLINE]Related articles

Aging results in paradoxical susceptibility of fat cell progenitors to lipotoxicity.

Guo W, Pirtskhalava T, Tchkonia T, Xie W, Thomou T, Han J, Wang T, Wong S, Cartwright A, Hegardt FG, Corkey BE, Kirkland JL.

Am J Physiol Endocrinol Metab. 2007 Apr;292(4):E1041-51. Epub 2006 Dec 5.PMID: 17148751 [PubMed - indexed for MEDLINE]Related articlesFree article

CPT I overexpression protects L6E9 muscle cells from fatty acid-induced insulin resistance.

Sebastián D, Herrero L, Serra D, Asins G, Hegardt FG.

Am J Physiol Endocrinol Metab. 2007 Mar;292(3):E677-86. Epub 2006 Oct 24.PMID: 17062841 [PubMed - indexed for MEDLINE]Related articlesFree article

Mutagenesis of specific amino acids converts carnitine acetyltransferase into carnitine palmitoyltransferase.

Cordente AG, López-Viñas E, Vázquez MI, Gómez-Puertas P, Asins G, Serra D, Hegardt FG.

Biochemistry. 2006 May 16;45(19):6133-41.PMID: 16681386 [PubMed - indexed for MEDLINE]Related articles

Refining the diagnosis of mitochondrial HMG-CoA synthase deficiency.

Aledo R, Mir C, Dalton RN, Turner C, Pié J, Hegardt FG, Casals N, Champion MP.

J Inherit Metab Dis. 2006 Feb;29(1):207-11.PMID: 16601895 [PubMed - indexed for MEDLINE]Related articles

A single-residue mutation, G203E, causes 3-hydroxy-3-methylglutaric aciduria by occluding the substrate channel in the 3D structural model of HMG-CoA lyase.

Mir C, Lopez-Viñas E, Aledo R, Puisac B, Rizzo C, Dionisi-Vici C, Deodato F, Pié J, Gomez-Puertas P, Hegardt FG, Casals N.

J Inherit Metab Dis. 2006 Feb;29(1):64-70.PMID: 16601870 [PubMed - indexed for MEDLINE]Related articles

Novel effect of C75 on carnitine palmitoyltransferase I activity and palmitate oxidation.

Bentebibel A, Sebastián D, Herrero L, López-Viñas E, Serra D, Asins G, Gómez-Puertas P, Hegardt FG.

Biochemistry. 2006 Apr 11;45(14):4339-50.PMID: 16584169 [PubMed - indexed for MEDLINE]Related articles

Alteration of the malonyl-CoA/carnitine palmitoyltransferase I interaction in the beta-cell impairs glucose-induced insulin secretion.

Herrero L, Rubí B, Sebastián D, Serra D, Asins G, Maechler P, Prentki M, Hegardt FG.

Diabetes. 2005 Feb;54(2):462-71.PMID: 15677504 [PubMed - indexed for MEDLINE]Related articlesFree article

Molecular basis of 3-hydroxy-3-methylglutaric aciduria.

Pie J, Casals N, Puisac B, Hegardt FG.

J Physiol Biochem. 2003 Dec;59(4):311-21.PMID: 15164951 [PubMed - indexed for MEDLINE]Related articles

Redesign of carnitine acetyltransferase specificity by protein engineering.

Cordente AG, López-Viñas E, Vázquez MI, Swiegers JH, Pretorius IS, Gómez-Puertas P, Hegardt FG, Asins G, Serra D.

J Biol Chem. 2004 Aug 6;279(32):33899-908. Epub 2004 May 21.PMID: 15155769 [PubMed - indexed for MEDLINE]Related articlesFree article

Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal.

Morillas M, López-Viñas E, Valencia A, Serra D, Gómez-Puertas P, Hegardt FG, Asins G.

Biochem J. 2004 May 1;379(Pt 3):777-84.PMID: 14711372 [PubMed - indexed for MEDLINE]Related articlesFree article

Structural (betaalpha)8 TIM barrel model of 3-hydroxy-3-methylglutaryl-coenzyme A lyase.

Casals N, Gómez-Puertas P, Pié J, Mir C, Roca R, Puisac B, Aledo R, Clotet J, Menao S, Serra D, Asins G, Till J, Elias-Jones AC, Cresto JC, Chamoles NA, Abdenur JE, Mayatepek E, Besley G, Valencia A, Hegardt FG.

J Biol Chem. 2003 Aug 1;278(31):29016-23. Epub 2003 May 13.PMID: 12746442 [PubMed - indexed for MEDLINE]Related articlesFree article

Identification of conserved amino acid residues in rat liver carnitine palmitoyltransferase I critical for malonyl-CoA inhibition. Mutation of methionine 593 abolishes malonyl-CoA inhibition.

Morillas M, Gómez-Puertas P, Bentebibel A, Sellés E, Casals N, Valencia A, Hegardt FG, Asins G, Serra D.

J Biol Chem. 2003 Mar 14;278(11):9058-63. Epub 2002 Dec 23.PMID: 12499375 [PubMed - indexed for MEDLINE]Related articlesFree article

Impaired ketogenesis is a major mechanism for disturbed hepatic fatty acid metabolism in rats with long-term cholestasis and after relief of biliary obstruction.

Lang C, Berardi S, Schäfer M, Serra D, Hegardt FG, Krähenbühl L, Krähenbühl S.

J Hepatol. 2002 Nov;37(5):564-71.PMID: 12399220 [PubMed - indexed for MEDLINE]Related articles

The diagnosis of mitochondrial HMG-CoA synthase deficiency.

Zschocke J, Penzien JM, Bielen R, Casals N, Aledo R, Pié J, Hoffmann GF, Hegardt FG, Mayatepek E.

J Pediatr. 2002 Jun;140(6):778-80.PMID: 12072887 [PubMed - indexed for MEDLINE]Related articles

Adenovirus-mediated overexpression of liver carnitine palmitoyltransferase I in INS1E cells: effects on cell metabolism and insulin secretion.

Rubí B, Antinozzi PA, Herrero L, Ishihara H, Asins G, Serra D, Wollheim CB, Maechler P, Hegardt FG.

Biochem J. 2002 May 15;364(Pt 1):219-26.PMID: 11988095 [PubMed - indexed for MEDLINE]Related articlesFree article

Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: mutational analysis of a malonyl-CoA affinity domain.

Morillas M, Gómez-Puertas P, Rubí B, Clotet J, Ariño J, Valencia A, Hegardt FG, Serra D, Asins G.

J Biol Chem. 2002 Mar 29;277(13):11473-80. Epub 2002 Jan 14.PMID: 11790793 [PubMed - indexed for MEDLINE]Related articlesFree article

Structural model of the catalytic core of carnitine palmitoyltransferase I and carnitine octanoyltransferase (COT): mutation of CPT I histidine 473 and alanine 381 and COT alanine 238 impairs the catalytic activity.

Morillas M, Gómez-Puertas P, Roca R, Serra D, Asins G, Valencia A, Hegardt FG.

J Biol Chem. 2001 Nov 30;276(48):45001-8. Epub 2001 Sep 11.PMID: 11553629 [PubMed - indexed for MEDLINE]Related articlesFree article

Genetic basis of mitochondrial HMG-CoA synthase deficiency.

Aledo R, Zschocke J, Pié J, Mir C, Fiesel S, Mayatepek E, Hoffmann GF, Casals N, Hegardt FG.

Hum Genet. 2001 Jul;109(1):19-23.PMID: 11479731 [PubMed - indexed for MEDLINE]Related articles

GAD65-mediated glutamate decarboxylation reduces glucose-stimulated insulin secretion in pancreatic beta cells.

Rubi B, Ishihara H, Hegardt FG, Wollheim CB, Maechler P.

J Biol Chem. 2001 Sep 28;276(39):36391-6. Epub 2001 Jul 25.PMID: 11473118 [PubMed - indexed for MEDLINE]Related articlesFree article

Localization of an exonic splicing enhancer responsible for mammalian natural trans-splicing.

Caudevilla C, Codony C, Serra D, Plasencia G, Román R, Graessmann A, Asins G, Bach-Elias M, Hegardt FG.

Nucleic Acids Res. 2001 Jul 15;29(14):3108-15.PMID: 11452036 [PubMed - indexed for MEDLINE]Related articlesFree article

Modulation in vitro of H-ras oncogene expression by trans-splicing.

Codony C, Guil S, Caudevilla C, Serra D, Asins G, Graessmann A, Hegardt FG, Bach-Elias M.

Oncogene. 2001 Jun 21;20(28):3683-94.PMID: 11439331 [PubMed - indexed for MEDLINE]Related articlesFree article

Low activity of mitochondrial HMG-CoA synthase in liver of starved piglets is due to low levels of protein despite high mRNA levels.

Barrero MJ, Alho CS, Ortiz JA, Hegardt FG, Haro D, Marrero PF.

Arch Biochem Biophys. 2001 Jan 15;385(2):364-71.PMID: 11368018 [PubMed - indexed for MEDLINE]Related articles

Developmental changes in carnitine octanoyltransferase gene expression in intestine and liver of suckling rats.

Miliar A, Serra D, Casaroli R, Vilaró S, Asins G, Hegardt FG.

Arch Biochem Biophys. 2001 Jan 15;385(2):283-9.PMID: 11368009 [PubMed - indexed for MEDLINE]Related articles

Post-transcriptional regulation of rat carnitine octanoyltransferase.

Hegardt FG, Bach M, Asins G, Caudevilla C, Morillas M, Codony C, Serra D.

Biochem Soc Trans. 2001 May;29(Pt 2):316-20.PMID: 11356174 [PubMed - indexed for MEDLINE]Related articles

Pig liver carnitine palmitoyltransferase I, with low Km for carnitine and high sensitivity to malonyl-CoA inhibition, is a natural chimera of rat liver and muscle enzymes.

Nicot C, Hegardt FG, Woldegiorgis G, Haro D, Marrero PF.

Biochemistry. 2001 Feb 20;40(7):2260-6.PMID: 11329295 [PubMed - indexed for MEDLINE]Related articles

Differential expression of cytosolic and mitochondrial 3-hydroxy-3-methylglutaryl CoA synthases during adipocyte differentiation.

Lopez JM, Hegardt FG, Haro D.

Mol Cell Biochem. 2001 Jan;217(1-2):57-66.PMID: 11269666 [PubMed - indexed for MEDLINE]Related articles

3-Hydroxy-3-methylglutaryl coenzyme A synthase-1 of Blattella germanica has structural and functional features of an active retrogene.

Casals N, Buesa C, Marrero PF, Belles X, Hegardt FG.

Insect Biochem Mol Biol. 2001 Mar 15;31(4-5):425-33.PMID: 11222952 [PubMed - indexed for MEDLINE]Related articles

Inhibition by etomoxir of rat liver carnitine octanoyltransferase is produced through the co-ordinate interaction with two histidine residues.

Morillas M, Clotet J, Rubí B, Serra D, Ariño J, Hegardt FG, Asins G.

Biochem J. 2000 Oct 15;351 Pt 2:495-502.PMID: 11023836 [PubMed - indexed for MEDLINE]Related articlesFree article

Contribution of steroidogenic factor 1 to the regulation of cholesterol synthesis.

Mascaró C, Nadal A, Hegardt FG, Marrero PF, Haro D.

Biochem J. 2000 Sep 15;350 Pt 3:785-90.PMID: 10970793 [PubMed - indexed for MEDLINE]Related articlesFree article

Mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase and carnitine palmitoyltransferase II as potential control sites for ketogenesis during mitochondrion and peroxisome proliferation.

Madsen L, Garras A, Asins G, Serra D, Hegardt FG, Berge RK.

Biochem Pharmacol. 1999 May 1;57(9):1011-9.PMID: 10796071 [PubMed - indexed for MEDLINE]Related articles

Processing of carnitine octanoyltransferase pre-mRNAs by cis and trans-splicing.

Caudevilla C, Serra D, Miliar A, Codony C, Asins G, Bach M, Hegardt FG.

Adv Exp Med Biol. 1999;466:95-102.PMID: 10709632 [PubMed - indexed for MEDLINE]Related articles

Characterization of a response element for peroxisomal proliferator activated receptor (PPRE) in human muscle-type carnitine palmitoyltransferase I.

Mascaró C, Acosta E, Ortiz JA, Rodríguez JC, Marrero PF, Hegardt FG, Haro D.

Adv Exp Med Biol. 1999;466:79-85. Review. No abstract available. PMID: 10709630 [PubMed - indexed for MEDLINE]Related articles

Sterol regulatory element binding protein-mediated effect of fluvastatin on cytosolic 3-hydroxy-3-methylglutaryl-coenzyme A synthase transcription.

Mascaró C, Ortiz JA, Ramos MM, Haro D, Hegardt FG.

Arch Biochem Biophys. 2000 Feb 15;374(2):286-92.PMID: 10666309 [PubMed - indexed for MEDLINE]Related articles

Identification of the two histidine residues responsible for the inhibition by malonyl-CoA in peroxisomal carnitine octanoyltransferase from rat liver.

Morillas M, Clotet J, Rubí B, Serra D, Asins G, Ariño J, Hegardt FG.

FEBS Lett. 2000 Jan 21;466(1):183-6.PMID: 10648838 [PubMed - indexed for MEDLINE]Related articles

Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase promoter contains a CREB binding site that regulates cAMP action in Caco-2 cells.

Eggers A, Caudevilla C, Asins G, Hegardt FG, Serra D.

Biochem J. 2000 Jan 15;345 Pt 2:201-6.PMID: 10620495 [PubMed - indexed for MEDLINE]Related articlesFree article

Mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase and carnitine palmitoyltransferase II are potential control sites of hepatic ketogenesis under conditions of peroxisome proliferation.

Berge RK, Garras A, Asins G, Serra D, Hegardt FG, Madsen L.

Lipids. 1999;34 Suppl:S163. No abstract available. PMID: 10419135 [PubMed - indexed for MEDLINE]Related articles

Mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase: a control enzyme in ketogenesis.

Hegardt FG.

Biochem J. 1999 Mar 15;338 ( Pt 3):569-82. Review.PMID: 10051425 [PubMed - indexed for MEDLINE]Related articlesFree article

Transcriptional regulation of mitochondrial HMG-CoA synthase in the control of ketogenesis.

Hegardt FG.

Biochimie. 1998 Oct;80(10):803-6. Review.PMID: 9893938 [PubMed - indexed for MEDLINE]Related articles

Isolation of pig mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase gene promoter: characterization of a peroxisome proliferator-responsive element.

Ortiz JA, Mallolas J, Nicot C, Bofarull J, Rodríguez JC, Hegardt FG, Haro D, Marrero PF.

Biochem J. 1999 Jan 15;337 ( Pt 2):329-35.PMID: 9882632 [PubMed - indexed for MEDLINE]Related articlesFree article

Two missense point mutations in different alleles in the 3-hydroxy-3-methylglutaryl coenzyme A lyase gene produce 3-hydroxy-3-methylglutaric aciduria in a French patient.

Zapater N, Pié J, Lloberas J, Rolland MO, Leroux B, Vidailhet M, Divry P, Hegardt FG, Casals N.

Arch Biochem Biophys. 1998 Oct 15;358(2):197-203.PMID: 9784232 [PubMed - indexed for MEDLINE]Related articles