Visualitzant Ciències de la Salut per autor/a "Valencia, Alfonso"
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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, Montserrat; Gómez-Puertas, Paulino; Bentebibel, Assia; Sellés, Eva; Casals i Farré, Núria; Valencia, Alfonso; Hegardt, Fausto G.; Asins, Guillermina; Serra, Dolors (Journal of Biological Chemistry, 2003-03-14)Carnitine palmitoyltransferase (CPT) I, which catalyzes the conversion of palmitoyl-CoA to palmitoylcarnitine facilitating its transport through the mitochondrial membranes, is inhibited by malonyl-CoA. ... -
Structural (betaalpha) 8 TIM barrel model of 3-hydroxy-3-methylglutaryl-coenzyme A lyase
Casals i Farré, Núria; Gómez-Puertas, Paulino; Pié, Juan; Mir, Cecilia; Roca, Ramón; Puisac, Beatriz; Aledo, Rosa; Clotet Erra, Josep; Menao, Sebastián; Serra, Dolors; Asins, Guillermina; Till, Jacqueline; Elias-Jones, Alun C.; Cresto, Juan C.; Chamoles, Nestor A.; Abdenur, Jose E.; Mayatepek, Ertan; Besley, Guy; Valencia, Alfonso; Hegardt, Fausto G. (Journal of Biological Chemistry, 2003)This study describes three novel homozygous missense mutations (S75R, S201Y, and D204N) in the 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) lyase gene, which caused 3-hydroxy-3-methylglutaric aciduria in ...