López-Viñas E - Search Results

1

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. Carrasco P, et al. Mol Genet Metab. 2007 Jun;91(2):120-7. doi: 10.1016/j.ymgme.2007.03.007. Epub 2007 Apr 24. Mol Genet Metab. 2007. PMID: 17459752

2

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. Mir C, et al. J Inherit Metab Dis. 2006 Feb;29(1):64-70. doi: 10.1007/s10545-006-0138-x. J Inherit Metab Dis. 2006. PMID: 16601870

3

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. Pié J, et al. Mol Genet Metab. 2007 Nov;92(3):198-209. doi: 10.1016/j.ymgme.2007.06.020. Epub 2007 Aug 9. Mol Genet Metab. 2007. PMID: 17692550 Review.

4

Characterization of a novel HMG-CoA lyase enzyme with a dual location in endoplasmic reticulum and cytosol.

Arnedo M, Menao S, Puisac B, Teresa-Rodrigo ME, Gil-Rodríguez MC, López-Viñas E, Gómez-Puertas P, Casals N, Casale CH, Hegardt FG, Pié J. Arnedo M, et al. Among authors: lopez vinas e. J Lipid Res. 2012 Oct;53(10):2046-2056. doi: 10.1194/jlr.M025700. Epub 2012 Jul 30. J Lipid Res. 2012. PMID: 22847177 Free PMC article.

5

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. Morillas M, et al. Biochem J. 2004 May 1;379(Pt 3):777-84. doi: 10.1042/BJ20031373. Biochem J. 2004. PMID: 14711372 Free PMC article.

6

Skipping of exon 2 and exons 2 plus 3 of HMG-CoA lyase (HL) gene produces the loss of beta sheets 1 and 2 in the recently proposed (beta-alpha)8 TIM barrel model of HL.

Puisac B, López-Viñas E, Moreno S, Mir C, Pérez-Cerdá C, Menao S, Lluch D, Pié A, Gómez-Puertas P, Casals N, Ugarte M, Hegardt F, Pié J. Puisac B, et al. Biophys Chem. 2005 Apr 1;115(2-3):241-5. doi: 10.1016/j.bpc.2004.12.031. Epub 2005 Jan 6. Biophys Chem. 2005. PMID: 15752612

7

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. Menao S, et al. Hum Mutat. 2009 Mar;30(3):E520-9. doi: 10.1002/humu.20966. Hum Mutat. 2009. PMID: 19177531

8

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. Bentebibel A, et al. Biochemistry. 2006 Apr 11;45(14):4339-50. doi: 10.1021/bi052186q. Biochemistry. 2006. PMID: 16584169

9

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. Cordente AG, et al. J Biol Chem. 2004 Aug 6;279(32):33899-908. doi: 10.1074/jbc.M402685200. Epub 2004 May 21. J Biol Chem. 2004. PMID: 15155769 Free article.

10

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. Cordente AG, et al. Biochemistry. 2006 May 16;45(19):6133-41. doi: 10.1021/bi0602664. Biochemistry. 2006. PMID: 16681386

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