Publicaciones por líneas de investigación actuales 

Publicaciones por temas de investigación-historial del grupo

1. Estudio molecular de los genes del transporte de ácidos grasos en la mitocondria: Carnitina palmitoil transferasa I, Carnitina octanoil transferasa y Carnitina Acetil transferasa. Modelos de estructura tridimensional y funcionalidad
   
2. Mecanismos moleculares de acción de la carnitina palmitoil transferasa I en la secreción de insulina en la célula beta del páncreas y disminución de la resistencia a la insulina en células musculares.
   
3. Análisis moleculaesr de las mutaciones que producen enfemedades hereditarias en las enzimas de la cetogénesis: HMG-CoA sintasa mitocondrial y HMG-CoA liasa.
   
4. Aspectos moleculares de procesos de trans-splicing en mamíferos
   

Publicaciones apartado 1. Estudio molecular de los genes del transporte de ácidos grasos a la mitocondria: Carnitina palmitoil transferasa I, Carnitina octanoil transferasa y Carnitina Acetil transferasa. Modelos de estructura tridimensional y funcionalidad

• Asins, G., Serra, D., Arias, G. & Hegardt, F.G.
  Developmental changes in Carnitine Palmitoyl Transferases I and II gene expression in intestine and liver of suckling rats
  Biochemical Journal (1995) 306, 379-38. Abstract
• Arias, G., Matas, R., Asins, G., Hegardt, F.G. & Serra, D.
  The effect of fasting and insulin treatment on carnitine palmitoyltransferase I and mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase mRNA levels in liver from suckling rats
  Biochemical Society Transactions (1997) 23, S493. Abstract
• Mascaró, C., Acosta, E., Ortiz, JA., Marrero, PF., Hegardt, F.G. y Haro, D.
  Control of human type Carnitine Palmitoyl Transferase I gene transcription by peroxisome proliferator-activated receptor
  Journal of Biological Chemistry (1998) 273, 8560-8563. Abstract
• Berge, R.K.; Garras, A.; Asins, G.; Serra, D.; Hegardt, F.G.; Madsen, L.
  Mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase and carnitine palmitoyltransferase II are potential control sites of hepatic ketogenesis under conditions of peroxisome proliferation
  Lipids (1999) 34, S163-163.
• Mascaró, C., Acosta, E., Ortiz, J.A., Rodriguez, J.C., Marrero, P.F., Hegardt, F.G. and Haro, D.
  Characterization of a response element for peroxisomal proliferator activated receptor (PPRE) in human muscle-type carnitine palmitoyltransferase I
  Advances in Experimental Medicine and Biology (1999) 466, 79-85.
• Morillas, M., Clotet, J., Rubí, B., Serra, D., Asins, G., Ariño, J. y Hegardt, F.G.
  Identification of the two histidine residues responsible for the inhibition by malonyl-CoA in peroxisomal carnitine octanoyltransferase from rat liver
  Febs Letters (2000) 466, 183-186.Abstract
• Morillas, M., Clotet, J. Rubí, B., Serra, D., Ariño, J., Hegardt, F.G. y Asins, G.
  Inhibition by etomoxir of rat liver carnitine octanoyltransferase is produced through the coordinate interaction with two histidine residues
  Biochemical Journal. (2000) 351, 495-502 Abstract
• Miliar, A., Serra, D., Casaroli, R., Vilaró, S., Asins, G. y Hegardt, F.G.
  Developmental changes in carnitine octanoyltransferase gene expression in intestine and liver of suckling rats
  Archives of Biochemistry and Biophysics (2001) 385, 283-289. Abstract
• Madsen, L., Garras, A., Asins, G., Serra, D., Hegardt, F.G. y Berge, R.
  Mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase and carnitine palmitoyltransferase II are potential control sites of hepatic ketogenesis under conditions of peroxisome proliferation
  Biochemical Pharmacology (1999) 57, 1011-1019.
• Nicot, C., Hegardt, F.G. Woldegiorgis, G., Haro, D., Marrero, P.
  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
  Biochemistry (2001) 40, 1351-1357. Abstract
• Morillas, M., Gómez-Puertas, P., Roca, R., Serra, D., Asins, G., Valencia, A. y Hegardt, F.G.
  Structural model of the catalytic core of carnitine palmitoyltransferase I and carnitine octanoyltransferase
  Journal of Biological Chemistry (2001) 276, 45001-45008. Abstract
• Morillas, M., Gómez-Puertas, P., Rubí, B., Clotet, J., Ariño, J., Valencia, A., Hegardt, F.G., Serra, D. y Asins, G.
  Structural model of a malonyl-CoA binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I. Mutational analysis of a malonyl-CoA affinity domain.
  Journal of Biological Chemistry (2002) 277, 11473-11480. Abstract
• Morillas, M., Gómez-Puertas, P., Bentebibel, A., Sellés, E., Casals, N., Valencia, A., Hegardt, F.G.,
  Asins, G. y Serra, D.
  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.
  Journal of Biological Chemistry (2003) 278, 9058-9063. Abstract
• Cordente AG, Lopez-Vinas E, Vazquez MI, Swiegers JH, Pretorius IS, Gomez-Puertas P, Hegardt FG, Asins G, Serra D Redesign of carnitine acetyltransferase specificity by protein engineering. Modification of methionine564 broadens the specificity to longer acyl-CoAs as substrates. J Biol Chem. 2004 May 21 Abstract

  

  Publicaciones apartado 2. Mecanismos moleculares de acción de la carnitina palmitoil transferasa I en la secreción de insulina en la célula beta del páncreasy disminución de la resistencia a la insulina en célules musculares

• Rubi B, Ishihara H, Hegardt FG, Wollheim CB, Maechler P.
  GAD65-mediated glutamate decarboxylation reduces glucose-stimulated insulin secretion in pancreatic beta cells.
  Journal of Biological Chemistry (2001) 276, 36391-36396. Abstract
• Rubí, B., Antinozzi, P.A., Herrero, L., Ishihara, H., Asins, G., Serra, D., Wollheim, C.B., Maechler, P. y Hegardt, F.G.
  Adenovirus-mediated overexpression of liver carnitine palmitoyltransferase I in INS1E cells. Effects on cell metabolism and insulin secretion
  Biochemical Journal (2002) 364, 219-226. Abstract

  
  Publicaciones apartado 3. Análisis molecular de las mutaciones causantes de enfermedades hereditarias en las enzimas de la cetogénesis: HMG-CoA sintasa mitocondrial y HMG-CoA liasa.

• Buesa, C., Pie, J., Barceló, A., Casals, N., Mascaró, C., Casale, C.H., Haro, D., Duran, M., Smeitink, J.A.M & Hegardt, F.G.
  Aberrantly spliced mRNAs of the 3-Hydroxy-3-methylglutaryl coenzyme A lyase (HL) gene with a donor splice site point mutation produce hereditary HL deficiency
  Journal of Lipid Research (1996) 37, 2420-2432. Abstract
• Pié, P., Casals, N., Casale, C.H., Buesa, C., Mascaró, C., Barceló, A., Rolland, M. O., Zabot, M.T., Haro, D., Eyskens, F., Divry, P., & Hegardt, F.G.
  A nonsense mutation in the 3-hydroxy-3-methylglutaryl coenzyme A lyase gene produces exon skipping in two patients of different origin with HL deficiency
  Biochemical Journal (1997) 323, 329-335. Abstract
• Casals, N., Pié, J., Casale, C.H., Zapater, N., Ribes, A., Castro-Gago, M., Rodriguez-Segade, S., Wanders, R.J.A. y Hegardt, F.G.
  A two-base deletion in exon 6 of the 3-hydroxy-3-methylglutaryl coenzyme A lyase (HL) gene producing the skipping of exons 5 and 6 determines 3-hydroxy 3-methylglutaric aciduria
  Journal of Lipid Research. (1997) 38, 2303-2313. Abstract
• Casale, C.H., Casals, N., Pié, J., Zapater, N., Pérez-Cerdá, C., Merinero, B., Martínez-Pardo, M., García-Peñas, J.J., García-Gonzalez, J.M., Lama, R., Poll-The, B.T., Smeitink, J.A.M., Wanders, R.J.A., Ugarte, M., and Hegardt, F.G.
  A nonsense mutation in the exon 2 of the 3-hydroxy-3-methylglutaryl CoA lyase (HL) gene producing three mature mRNAs is the main cause of 3-hydroxy-3-methylglutaric aciduria in european mediterranean patients.
  Archives of Biochemistry and Biophysics (1998) 349, 129-137. Abstract
• Zapater, N., Pié, J., Lloberas, J., Rolland, M.O., Leroux, B., Vidailhet, M., Divry, P., Hegardt, F.G. y Casals, N.
  Two missense point mutations in different alleles in the 3-hydroxy-3-methylglutaryl coenzyme A lyase gene produce the 3-hydroxy-3-methylglutaric aciduria
  Archives of Biochemistry and Biophysics (1998) 358, 197-203. Abstract
• Hegardt, F.G.
  La aciduria 3-Hidroxi-3-metilglutàrica: Una malaltia molecular per deficiència de 3-Hidroxi-3-metilglutaril coenzim A liasa
  Treballs de la Societat Catalana de Biologia (1999) 49, 133-150.
• Aledo, R., Zschocke, J., Pié, J., Mir, C., Fiesel, S., Mayatepek, E., Hoffmann, G.F., Casals, N. y Hegardt, F.G.
  The genetic basis of mitochondrial HMG-CoA synthase deficiency
  Human Genetics (2001) 109, 19-23. Abstract
• Zschocke, J., Penzien, J.M., Bielen, R., Casals, N., Aledo, R., Pié, J., Hoffmann, G.F., Hegardt, F.G. y Mayatepek, E.
  The diagnosis of mitochondrial HMG-CoA synthase deficiency
  The Journal of Pediatrics (2002) 140, 778-780. Abstract
• 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, A.C., Cresto, J.C., Chamoles, N.A., Abdenur, J.E., Mayatepek, E., Besley, G., Valencia, A. y Hegardt, F.G.
  Structural TIM barrel model of 3-Hydroxy-3-methylglutaryl Coenzyme A lyase.
  Journal of Biological Chemistry (2003) 278, 29016-29023. Abstract

Publicaciones apartado .4. Aspectos moleculares de procesos de trans-splicing en mamíferos

• Caudevilla, C., Serra, D., Miliar, A., Codony, C., Asins, G., Bach, M. y Hegardt F.G. Natural trans-splicing in the carnitine octanoyl transferase pre-mRNAs in rat liver.
  Proceedings of the National Academy of Sciences USA. (1998) 95, 12185-12190. Abstract
• Caudevilla, C., Serra, D., Miliar, A., Codony, C., Asins, G., Bach, M. y Hegardt, F.G. Processing of carnitine octanoiltransferase pre-mRNAs by cis- and transsplicing. Advances in Experimental Medicine and Biology (1999) 466, 95-102. Abstract
• Hegardt, F.G., Bach, M., Asins, G., Caudevilla, C., Morillas, M. Codony, C y Serra, D. Post-transcriptional regulation of rat carnitine octanoyltransferase
  Biochemical Society Transactions (2001) 29, 316-320. Abstract
• Codony, C., Guil, S., Caudevilla, C., Serra, D., Asins, G., Graessmann, A., Hegardt, F.G. y Bach-Elias, M. Modulation in vitro of H-ras oncogene expression by trans-splicing.
  Oncogene (2001) 20, 3683-3694. Abstract
• Caudevilla, C., Codony, C., Serra, D., Plasencia, G., Román, R., Graessmann, A, Asins, G., Bach-Elias, M. y Hegardt, F.G. Localization of an exonic splicing enhancer responsible for mammalian natural trans-splicing.
  Nucleic Acids Research (2001) 29, 3108-3115. Abstract

  

CONJUNTO DE PUBLICACIONES POR TEMAS DE INVESTIGACION (historial del grupo) 

Trabajos de Investigación desde 1990 según las líneas de investigación siguientes:

1. HMG-CoA sintasa mitocondrial
2. HMG-CoA sintasa citosólica
3. Carnitina Palmitoil transferasas
4. Estudios en Blattella germánica
5. Deficiencias hereditarias en HMG-CoA liasa y HMG-CoA sintasa
6. Trans-splicing en carnitina transferases de hígado de rata
7. Secreción de insulina por la célula beta del páncreas
8. Colestasis

1. HMG-CoA SINTASA MITOCONDRIAL

Estructura Génica

• Ayté, J., Gil-Gómez, G., Haro, D., Marrero, P.F. & Hegardt, F.G.
  Rat mitochondrial and cytosolic HMG-CoA synthases are encoded by two different genes
  Proceedings of the National Academy of Sciences USA (1990) 87, 3874-3878
• Gil-Gomez, G., Ayté, J. & Hegardt, F.G.
  The rat mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase gene contains elements that mediate its multihormonal regulation and tissue-specificity
  European Journal of Biochemistry (1993) 213, 773-779
• Ayté, J., Gil-Gómez, G. & Hegardt, F.G.
  Structural characterization of the 3´ non-coding region of the gene encoding rat mitochondrial 3-hydroxy 3-methylglutaryl coenzyme A synthase
  Gene (1993) 123, 267-270
• Mascaró, C., Buesa, C., Ortiz, J.A., Haro, D. & Hegardt, F.G.
  Molecular cloning and tissue expression of human mitochondrial 3-Hydroxy-3-methylglutaryl CoA synthase
  Archives of Biochemistry and Biophysics (1995) 317, 385-
• Adams, S.H., Alho, C.S., Asins, G., Hegardt, F.G. y Marrero, P.F.
  Gene expression of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase in a poorly ketogenic mammal. Effect of starvation during neonatal period in the piglet
  Biochemical Journal (1997) 324, 65-73

Regulación de la Expresión Génica

• Casals, N., Roca, N., Guerrero, M., Gil-Gómez, G., Ayté, J., Ciudad, C.J. & Hegardt, F.G.
  Regulation of the expression of the mitochondrial 3-hydroxy 3-methylglutaryl coenzyme A synthase gene. Its role in control of ketogenesis.
  Biochemical Journal (1992) 283, 261-264
• Serra, D., Casals, N., Asins, G., Royo, T., Ciudad, C.J. & Hegardt, F.G.
  Regulation of mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase protein by starvation, fat feeding, and diabetes
  Archives of Biochemistry and Biophysics (1993) 307, 40-45
• Ayté, J., Gil-Gómez, G. & Hegardt, F.G.
  Methylation of the regulatory region of the mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase gene leads to its transcriptional inactivation
  Biochemical Journal (1993) 295, 807-812
• 
  Rodriguez, J.C., Gil-Gómez, G., Hegardt, F.G. & Haro, D.
  The peroxisome proliferator-activated receptor mediates the induction of the mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase gene by fatty acids
  Journal of Biological Chemistry (1994) 269, 18767-18772
• Royo, T., Pedragosa, M.J., Ayté, J., Gil-Gómez, G., Vilaró, S. & Hegardt, F.G.
  Immunolocalization of mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase in rat liver
  Journal of Cellular Physiology (1995) 162, 103-109
• Hegardt, F.G.
  Regulation of mitochondrial HMG-CoA synthase gene expression in liver and intestine from the rat
  Biochemical Society Transactions (1995) 23, 486-490
• Rodríguez, J.C., Ortiz, J.A., Hegardt, F.G. y Haro, D.
  The chicken ovalbumin upstream promoter transcription factor (COUP-TF) could act as a transcriptional activator or repressor of the mitochondrial 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) synthase gene
  Biochemical Journal (1997) 326, 587-592
• Rodriguez, J.C., Ortiz, J.A., Hegardt, F.G. and Haro, D.
  The Hepatocyte Nuclear Factor 4 (HFN-4) represses the mitochondrial HMG-CoA synthase gene
  Biochemical Biophysical Research Communications (1998) 242, 692-696
• Hegardt, F.G.
  Transcriptional regulation of mitochondrial HMG-CoA synthase in the control of ketogenesis
  Biochemie (1998) 80, 803-806
• Hegardt, F.G.
  Mitochondrial 3-Hydroxy-3-methylglutaryl coenzyme A synthase: A control site in ketogenesis
  Biochemical Journal (1999) 338, 569-582 Review Article
• Ortiz, J.A., Maiolas, J., Nicot, C., Bofarull, J., Rodriguez, J.C., Hegardt, F.G., Haro, D. y Marrero, P.F.
  Isolation of pig mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase gene promoter. Characterization of a peroxisome proliferator responsive element (PPRE)
  Biochemical Journal. (1999) 337, 329-335
• Eggers, A., Caudevilla, C., Asins, G., Hegardt, F.G. y Serra, D.
  The mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase promoter contains a CREB binding site that regulates cAMP action in Caco2 cells
  Biochemical Journal (2000) 345, 201-206
• Lang, C., Schafer, M., Serra, D., Hegardt, F.G., Krähenbühl, L. and Krähenbühl, S.
  Impaired hepatic farry acid oxidation in rats with short-term cholestasis: characterization and mechanism.
  Journal of Lipid Research (2001) 42, 22-30
• Barrero, M., Alho, C., Ortiz, J., Hegardt, F.G., Haro, D. y Marrero, P.
  Low activity of mitochondrial HMG-CoA synthase in liver of starved piglets is due to low levels of protein despite high mRNA levels.
  Archives of Biochemistry and Biophysics (2001) 385, 364-371

Animales Transgénicos

• Valera, A., Pelegrín, M., Asins, G., Fillat, C., Sabater, J., Pujol, A., Hegardt, F.G. & Bosch, F.
  Overexpression of mitochondrial 3-Hydroxy-3-methylglutaryl CoA synthase in transgenic mice causes hepatic hyperketogenesis
  Journal of Biological Chemistry (1994) 269, 6267-6270
• Serra, D., Fillat, C., Matas, R., Bosch, F., & Hegardt, F.G.
  Tissue-specific expression and dietary regulation of chimeric mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase/human growth hormone gene in transgenic mice
  Journal of Biological Chemistry (1996) 271, 7529-753

Neonatos

• Serra, D., Asins, G. & Hegardt, F.G.
  Ketogenic mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase gene expression in intestine and liver of suckling rats
  Archives of Biochemistry and Biophysics (1993) 301, 445-448
• Serra, D., Bellido, D., Asins, G., Arias, G., Vilaró, S. & Hegardt, F.G.
  The expression of mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase in neonatal rat intestine is under transcriptional control.
  European Journal of Biochemistry (1996) 237, 16-24
• Arias, G., Asins, G., Hegardt, F.G. y Serra, D.
  The effect of fasting/refeeding and insulin treatment on the regulatory genes of ketogenesis
  Archives of Biochemistry and Biophysics (1997) 340, 287-298
  
  Asins, G., Serra, D., Miliar, A., Caudevilla, C., Matas, R., Arias, G., & Hegardt, F.G.
  Developmental changes in the phosphoenolpyruvate carboxykinase gene expression in small intestine and liver of suckling rats
  Archives of Biochemistry and Biophysics (1996) 329, 82-86
• Arias, G., Asins, G., Hegardt, F.G. and Serra, D.
  The effect of dexamethasone treatment on the expression of the regulatory genes of ketogenesis in intestine and liver of suckling rats.
  Molecular and Cellular Biochemistry. (1998) 178, 325-333
• Miliar, A., Serra, D., Casaroli, R., Vilaró, S., Asins, G. y Hegardt, F.G.
  Developmental changes in carnitine octanoyltransferase gene expression in intestine and liver of suckling rats
  Archives of Biochemistry and Biophysics (2001) 385, 283-289

Regeneración Hepàtica

• Asins, G., Roca, J.L., Serra, D., Ayté, J., Gil-Gómez, G., Tauler, A., Bartrons, R., & Hegardt, F.G.
  Gene expression of enzymes regulating ketogenesis and fatty acid metabolism in regenerating rat liver
  Biochemical Journal (1994) 299, 65-69

Farmacología Bioquímica

• Asins, G., Serra, D. & Hegardt, F.G.
  The effect of etomoxir on the mRNA levels of enzymes involved in ketogenesis and cholesterogenesis in rat liver
  Biochemical Pharmacology (1994) 47, 1373-1379
• Hegardt, F.G., Serra, D. & Asins, G.
  Influence of etomoxir on the expression of several genes in liver, testis and heart
  General Pharmacology (1995) 26, 897-904
• Madsen, L., Garras, A., Asins, G., Serra, D., Hegardt, F.G. y Berge, R.
  Mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase and carnitine palmitoyltransferase II are potential control sites of hepatic ketogenesis under conditions of peroxisome proliferation
  Biochemical Pharmacology (1999) 57, 1011-1019
• Mascaró, C., Ortiz, J.A., Ramos, M.M., Haro, D. y Hegardt, F.G.
  SREBP-mediated effect of fluvastatin on cytosolic HMG-CoA synthase transcription
  Archives of Biochemistry and Biophysics (2000) 374, 286-292

Síntesis de Colesterol en células de ovario

• Royo, T., Pedragosa, M.J., Ayté, J. Gil-Gomez, G., Vilaró, S. & Hegardt, F.G.
  Testis and ovary express the gene for the ketogenic mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase
  Journal of Lipid Research (1993) 34, 867-874
• Ortiz, J.A., Gil-Gomez, G., Casaroli-Marano, R.P., Vilaró, S., Hegardt, F.G. & Haro, D.
  Transfection of the ketogenic mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase cDNA into Mev-1 cells corrects their auxotrophy for mevalonate
  Journal of Biological Chemistry (1994) 269, 28523-28526

  

2. HMG-CoA SINTASA CITOSOLICA

• Ayté J., Gil-Gomez, G. & Hegardt, F.G.
  Nucleotide sequence of a rat liver cDNA encoding the cytosolic 3-hydroxy 3-methylglutaryl coenzyme A synthase
  Nucleic Acids Research (1990) 18, 3642-3642
• Royo, T., Ayté, J., Albericio, F., Giralt, F., Haro, D. & Hegardt, F.G.
  Diurnal rhythm of rat liver cytosolic 3-hydroxy-3-methylglutaryl coenzyme A synthase. Correlation of mRNA levels with enzyme activity and protein
  Biochemical Journal (1991) 280, 61-64
• Royo, T., Haro, D. & Hegardt, F.G.
  Regulation of cytosolic 3-hydroxy 3-methylglutaryl coenzyme A synthase mRNA levels by L-triiodothyronine
  Biochemical Journal (1993) 289, 557-560
• Mascaró, C., Ortiz, J.A., Ramos, M.M., Haro, D. & Hegardt, F.G.
  SREBP-mediated effect of fluvastatin on cytosolic HMG-CoA synthase transcription
  Archives of Biochemistry and Biophysics (2000) 374, 286-292
• Mascaró, C., Nadal, A., Hegardt, F.G., Marrero, P.J. y Haro, D.
  Contribution of Steroidogenic Factor-1 to the regulation of cholesterol synthesis
  Biochemical Journal (2000) 350, 785-790
• Lopez, J.M., Hegardt, F.G. y Haro, D.
  Differential expression of cytosolic and mitochondrial 3-hydroxy-3-methylglutaryl CoA synthases during adipocyte differentiation
  Molecular and Cellular Biochemistry (2001) 217, 57-66

  

 

3. CARNITINA PALMITOIL TRANSFERASAS

• Asins, G., Serra, D., Arias, G. & Hegardt, F.G.
  Developmental changes in Carnitine Palmitoyl Transferases I and II gene expression in intestine and liver of suckling rats
  Biochemical Journal (1995) 306, 379-384
• Arias, G., Matas, R., Asins, G., Hegardt, F.G. & Serra, D.
  The effect of fasting and insulin treatment on carnitine palmitoyltransferase I and mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase mRNA levels in liver from suckling rats
  Biochemical Society Transactions (1995) 23, S493
• Mascaró, C., Acosta, E., Ortiz, JA., Marrero, PF., Hegardt, F.G. y Haro, D.
  Control of human type Carnitine Palmitoyl Transferase I gene transcription by peroxisome proliferator-activated receptor
  Journal of Biological Chemistry (1998) 273, 8560-8563
• Berge, R.K.; Garras, A.; Asins, G.; Serra, D.; Hegardt, F.G.; Madsen, L.
  Mitochondrial 3-hydroxy-3-methylglutaryl CoA synthase and carnitine palmitoyltransferase II are potential control sites of hepatic ketogenesis under conditions of peroxisome proliferation
  Lipids (1999) 34, S163-163
• Mascaró, C., Acosta, E., Ortiz, J.A., Rodriguez, J.C., Marrero, P.F., Hegardt, F.G. and Haro, D.
  Characterization of a response element for peroxisomal proliferator activated receptor (PPRE) in human muscle-type carnitine palmitoyltransferase I
  Advances in Experimental Medicine and Biology (1999) 466, 79-85
• Morillas, M., Clotet, J., Rubí, B., Serra, D., Asins, G., Ariño, J. y Hegardt, F.G.
  Identification of the two histidine residues responsible for the inhibition by malonyl-CoA in peroxisomal carnitine octanoyltransferase from rat liver
  Febs Letters (2000) 466, 183-186
  
  Morillas, M., Clotet, J. Rubí, B., Serra, D., Ariño, J., Hegardt, F.G. y Asins, G.
  Inhibition by etomoxir of rat liver carnitine octanoyltransferase is produced through the coordinate interaction with two histidine residues
  Biochemical Journal. (2000) 351, 495-502
• Miliar, A., Serra, D., Casaroli, R., Vilaró, S., Asins, G. y Hegardt, F.G.
  Developmental changes in carnitine octanoyltransferase gene expression in intestine and liver of suckling rats
  Archives of Biochemistry and Biophysics (2001) 385, 283-289
• Madsen, L., Garras, A., Asins, G., Serra, D., Hegardt, F.G. y Berge, R.
  Mitochondrial 3-hydroxy-3-methylglutaryl coenzyme A synthase and carnitine palmitoyltransferase II are potential control sites of hepatic ketogenesis under conditions of peroxisome proliferation
  Biochemical Pharmacology (1999) 57, 1011-1019
• Nicot, C., Hegardt, F.G. Woldegiorgis, G., Haro, D., Marrero, P.
  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
  Biochemistry (2001) 40, 1351-1357
• Hegardt, F.G., Bach, M., Asins, G., Caudevilla, C., Morillas, M. Codony, C y Serra, D.
  Post-transcriptional regulation of rat carnitine octanoyltransferase
  Biochemical Society Transactions (2001) 29, 316-320
• Morillas, M., Gómez-Puertas, P., Roca, R., Serra, D., Asins, G., Valencia, A. y Hegardt, F.G.
  Structural model of the catalytic core of carnitine palmitoyltransferase I and carnitine octanoyltransferase
  Journal of Biological Chemistry (2001) 276, 45001-45008
• Morillas, M., Gómez-Puertas, P., Rubí, B., Clotet, J., Ariño, J., Valencia, A., Hegardt, F.G., Serra, D. y Asins, G.
  Structural model of a malonyl-CoA binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I. Mutational analysis of a malonyl-CoA affinity domainJournal of Biological Chemistry (2002) 277, 11473-11480
• Morillas, M., Gómez-Puertas, P., Bentebibel, A., Sellés, E., Casals, N., Valencia, A., Hegardt, F.G.,
  Asins, G. y Serra, D.
  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.
  Journal of Biological Chemistry (2003) 278, 9058-9063

  

4. ESTUDIOS EN EL INSECTO BLATTELLA GERMÀNICA

• Martinez-Gonzalez, J., Buesa, C., Piulachs, M.D., Bellés, X. & Hegardt, F.G.
  Molecular cloning, developmental pattern and tissue expression of 3-hydroxy 3-methylglutaryl coenzyme A reductase of the cockroach Blattella germanica.
  European Journal of Biochemistry (1993) 213, 233-241
• Martinez-Gonzalez, J., Buesa, C., Piulachs, M.D., Bellés, X. & Hegardt, F.G.
  3-hydroxy 3-methylglutaryl coenzyme A synthase from the cockroach Blattella germanica: Cloning, expression, developmental pattern and tissue expression
  European Journal of Biochemistry (1993) 217, 691-699
• Martinez-Gonzalez, J. & Hegardt, F.G.
  Cytochrome c oxidase subunit I from the cockroach Blattella germanica: Cloning, developmental pattern and tissue expression
  Insect Biochemistry and Molecular Biology (1994) 24, 619-626
• Buesa, C., Martinez-Gonzalez, J., Casals, N., Haro, D., Piulachs, M.D., Bellés, X. & Hegardt, F.G.
  Blattella germanica has two HMG-CoA synthase genes. Both are regulated in the ovary during the gonadotrophic cycle
  Journal of Biological Chemistry (1994) 269, 11707-11713
• Martinez-Gonzalez, J. & Hegardt, F.G.
  Characterization of a cDNA encoding a cytosolic Peptidyl-Prolyl Cis-Trans isomerase from Blattella germanica
  European Journal of Biochemistry (1996) 234, 284-292
• Casals, N., Buesa, C., Piulachs, M.D., Cabañó, J., Marrero, P.F., Bellés, X. & Hegardt, F.G.
  Coordinated expression and activity of 3-hydroxy-3-methylglutaryl coenzyme A synthase and reductase in the fat body of Blattella germanica (L.) during vitellogenesis
  Insect Biochemistry and Molecular Biology (1996) 26, 837-843
• Casals, N., Martín, D., Buesa, C., Piulachs, M.D., Hegardt, F.G. and Bellés, X.
  Expression and activity of 3-hydroxy-3-methylglutaryl-CoA synthase and reductase in the fat body of ovariectomized and allatectomized Blattella germanica
  Physiological Entomology (1997) 22, 6-12
• Cabañó, J., Buesa, C., Hegardt, F.G. & Marrero, P.F.
  Catalytic properties of recombinant 3-hydroxy-3-methylglutaryl coenzyme A synthase-1 from Blattella germanica
  Insect Biochemistry and Molecular Biology (1997) 27, 499-505
• Casals, N., Buesa, C., Marrero, P.F., Bellés, X. Y Hegardt, F.G.
  3-Hydroxy-3-methylglutaryl-CoA synthase-1 of Blatella germanica has structural and functional features of an active retrogene
  Insect Biochemistry and Molecular Biology (2001) 31, 425-433

  

5. DEFICIENCIAS HEREDITARIAS EN HMG-CoA LIASA Y SINTASA

• Buesa, C., Pie, J., Barceló, A., Casals, N., Mascaró, C., Casale, C.H., Haro, D., Duran, M., Smeitink, J.A.M & Hegardt, F.G.
  Aberrantly spliced mRNAs of the 3-Hydroxy-3-methylglutaryl coenzyme A lyase (HL) gene with a donor splice site point mutation produce hereditary HL deficiency
  Journal of Lipid Research (1996) 37, 2420-2432
• Pié, P., Casals, N., Casale, C.H., Buesa, C., Mascaró, C., Barceló, A., Rolland, M. O., Zabot, M.T., Haro, D., Eyskens, F., Divry, P., & Hegardt, F.G.
  A nonsense mutation in the 3-hydroxy-3-methylglutaryl coenzyme A lyase gene produces exon skipping in two patients of different origin with HL deficiency
  Biochemical Journal (1997) 323, 329-335
• Casals, N., Pié, J., Casale, C.H., Zapater, N., Ribes, A., Castro-Gago, M., Rodriguez-Segade, S., Wanders, R.J.A. y Hegardt, F.G.
  A two-base deletion in exon 6 of the 3-hydroxy-3-methylglutaryl coenzyme A lyase (HL) gene producing the skipping of exons 5 and 6 determines 3-hydroxy 3-methylglutaric aciduria
  Journal of Lipid Research. (1997) 38, 2303-2313
• Casale, C.H., Casals, N., Pié, J., Zapater, N., Pérez-Cerdá, C., Merinero, B., Martínez-Pardo, M., García-Peñas, J.J., García-Gonzalez, J.M., Lama, R., Poll-The, B.T., Smeitink, J.A.M., Wanders, R.J.A., Ugarte, M., and Hegardt, F.G.
  A nonsense mutation in the exon 2 of the 3-hydroxy-3-methylglutaryl CoA lyase (HL) gene producing three mature mRNAs is the main cause of 3-hydroxy-3-methylglutaric aciduria in european mediterranean patients.
  Archives of Biochemistry and Biophysics (1998) 349, 129-137
• Zapater, N., Pié, J., Lloberas, J., Rolland, M.O., Leroux, B., Vidailhet, M., Divry, P., Hegardt, F.G. y Casals, N.
  Two missense point mutations in different alleles in the 3-hydroxy-3-methylglutaryl coenzyme A lyase gene produce the 3-hydroxy-3-methylglutaric aciduria
  Archives of Biochemistry and Biophysics (1998) 358, 197-203
• Hegardt, F.G.
  La aciduria 3-Hidroxi-3-metilglutàrica: Una malaltia molecular per deficiència de 3-Hidroxi-3-metilglutaril coenzim A liasa
  Treballs de la Societat Catalana de Biologia (1999) 49, 133-150
• Aledo, R., Zschocke, J., Pié, J., Mir, C., Fiesel, S., Mayatepek, E., Hoffmann, G.F., Casals, N. y Hegardt, F.G.
  The genetic basis of mitochondrial HMG-CoA synthase deficiency
  Human Genetics (2001) 109, 19-23
• Zschocke, J., Penzien, J.M., Bielen, R., Casals, N., Aledo, R., Pié, J., Hoffmann, G.F., Hegardt, F.G. y Mayatepek, E.
  The diagnosis of mitochondrial HMG-CoA synthase deficiency
  The Journal of Pediatrics (2002) 140, 778-780
• 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, A.C., Cresto, J.C., Chamoles, N.A., Abdenur, J.E., Mayatepek, E., Besley, G., Valencia, A. y Hegardt, F.G.
  Structural (Beta)8 TIM barrel model of 3-Hydroxy-3-methylglutaryl Coenzyme A lyase.
  Journal of Biological Chemistry (2003) 278, 29016-29023

  

6. CARNITINA OCTANOIL TRANSFERASA Y TRANS SPLICING

• Caudevilla, C., Serra, D., Miliar, A., Codony, C., Asins, G., Bach, M. y Hegardt F.G.
  Natural trans-splicing in the carnitine octanoyl transferase pre-mRNAs in rat liver
  Proceedings of the National Academy of Sciences USA. (1998) 95, 12185-12190
• Caudevilla, C., Serra, D., Miliar, A., Codony, C., Asins, G., Bach, M. y Hegardt, F.G.
  Processing of carnitine octanoiltransferase pre-mRNAs by cis- and trans-splicing
  Advances in Experimental Medicine and Biology (1999) 466, 95-102
• Codony, C., Guil, S., Caudevilla, C., Serra, D., Asins, G., Graessmann, A., Hegardt, F.G. y Bach-Elias,
  M.
  Modulation in vitro of H-ras oncogene expression by trans-splicing
  Oncogene (2001) 20, 3683-3694
• Caudevilla, C., Codony, C., Serra, D., Plasencia, G., Román, R., Graessmann, A, Asins, G., Bach-Elias, M. y Hegardt, F.G.
  Localization of an exonic splicing enhancer responsible for mammalian natural trans-splicing
  Nucleic Acids Research (2001) 29, 3108-3115

  

7. SECRECIÓN DE INSULINA PER LA CËLULA BETA DEL PANCREAS

• 
  Rubi B, Ishihara H, Hegardt FG, Wollheim CB, Maechler P.
  GAD65-mediated glutamate decarboxylation reduces glucose-stimulated insulin secretion in pancreatic beta cells.
  Journal of Biological Chemistry (2001) 276, 36391-36396
• Rubí, B., Antinozzi, P.A., Herrero, L., Ishihara, H., Asins, G., Serra, D., Wollheim, C.B., Maechler, P. y Hegardt, F.G.
  Adenovirus-mediated overexpression of liver carnitine palmitoyltransferase I in INS1E cells. Effects on cell metabolism and insulin secretion
  Biochemical Journal (2002) 364, 219-226

  

8. COLESTASIS

• Lang, C., Schafer, M., Serra, D., Hegardt, F.G., Krähenbühl, L. and Krähenbühl, S.
  Impaired hepatic farry acid oxidation in rats with short-term cholestasis: characterization and mechanism.
  Journal of Lipid Research (2001) 42, 22-30
• 
  Lang, C., Berardi, S., Schäfer, M., Serra, D., Hegardt, F.G., Krähenbühl, L. y Krähenbühl, S.
  Impaired ketogenesis is a major mechanism for disturbed hepatic fatty acid metabolism in rats with long-term cholestasis and after relief of biliary obstruction
  Journal of Hepatology (2002) 37, 564-571