139 related articles for article (PubMed ID: 12746442)
1. 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; 278(31):29016-23. PubMed ID: 12746442
[TBL] [Abstract][Full Text] [Related]
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
J Inherit Metab Dis; 2006 Feb; 29(1):64-70. PubMed ID: 16601870
[TBL] [Abstract][Full Text] [Related]
3. Crystal structure of human 3-hydroxy-3-methylglutaryl-CoA Lyase: insights into catalysis and the molecular basis for hydroxymethylglutaric aciduria.
Fu Z; Runquist JA; Forouhar F; Hussain M; Hunt JF; Miziorko HM; Kim JJ
J Biol Chem; 2006 Mar; 281(11):7526-32. PubMed ID: 16330550
[TBL] [Abstract][Full Text] [Related]
4. Crystal structures of two bacterial 3-hydroxy-3-methylglutaryl-CoA lyases suggest a common catalytic mechanism among a family of TIM barrel metalloenzymes cleaving carbon-carbon bonds.
Forouhar F; Hussain M; Farid R; Benach J; Abashidze M; Edstrom WC; Vorobiev SM; Xiao R; Acton TB; Fu Z; Kim JJ; Miziorko HM; Montelione GT; Hunt JF
J Biol Chem; 2006 Mar; 281(11):7533-45. PubMed ID: 16330546
[TBL] [Abstract][Full Text] [Related]
5. 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. PubMed ID: 17459752
[TBL] [Abstract][Full Text] [Related]
6. 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.
Casals N; Pié J; Casale CH; Zapater N; Ribes A; Castro-Gago M; Rodriguez-Segade S; Wanders RJ; Hegardt FG
J Lipid Res; 1997 Nov; 38(11):2303-13. PubMed ID: 9392428
[TBL] [Abstract][Full Text] [Related]
7. Evaluation of 3-hydroxy-3-methylglutaryl-coenzyme A lyase arginine-41 as a catalytic residue: use of acetyldithio-coenzyme A to monitor product enolization.
Tuinstra RL; Wang CZ; Mitchell GA; Miziorko HM
Biochemistry; 2004 May; 43(18):5287-95. PubMed ID: 15122894
[TBL] [Abstract][Full Text] [Related]
8. Investigation of conserved acidic residues in 3-hydroxy-3-methylglutaryl-CoA lyase: implications for human disease and for functional roles in a family of related proteins.
Tuinstra RL; Miziorko HM
J Biol Chem; 2003 Sep; 278(39):37092-8. PubMed ID: 12874287
[TBL] [Abstract][Full Text] [Related]
9. 3-hydroxy-3-methylglutaric aciduria: a new assay of 3-hydroxy-3-methylglutaryl-coa lyase using high performance liquid chromatography.
Gibson KM; Sweetman L; Nyhan WL; Page TM; Greene C; Cann HM
Clin Chim Acta; 1982 Dec; 126(2):171-81. PubMed ID: 6185253
[TBL] [Abstract][Full Text] [Related]
10. [3-hydroxy-3-methylglutaric aciduria and recurrent Reye-like syndrome].
Eirís J; Ribes A; Fernández-Prieto R; Rodríguez-García J; Rodríguez-Segade S; Castro-Gago M
Rev Neurol; 1998 Jun; 26(154):911-4. PubMed ID: 9658458
[TBL] [Abstract][Full Text] [Related]
11. Nucleotide sequence and expression in Escherichia coli of the 3-hydroxy-3-methylglutaryl coenzyme A lyase gene of Pseudomonas mevalonii.
Anderson DH; Rodwell VW
J Bacteriol; 1989 Dec; 171(12):6468-72. PubMed ID: 2687236
[TBL] [Abstract][Full Text] [Related]
12. 3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method.
Wanders RJ; Schutgens RB; Zoeters PH
Clin Chim Acta; 1988 Jan; 171(1):95-101. PubMed ID: 2450702
[TBL] [Abstract][Full Text] [Related]
13. 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
Biophys Chem; 2005 Apr; 115(2-3):241-5. PubMed ID: 15752612
[TBL] [Abstract][Full Text] [Related]
14. [3-hydroxy-3-methylglutaraciduria (case report of a female Turkish sisters with 3-hydroxy-3- methylglutaryl-Coenzyme A lyase deficiency].
Koling S; Kalhoff H; Schauerte P; Lehnert W; Diekmann L
Klin Padiatr; 2000; 212(3):113-6. PubMed ID: 10916782
[TBL] [Abstract][Full Text] [Related]
15. 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; 358(2):197-203. PubMed ID: 9784232
[TBL] [Abstract][Full Text] [Related]
16. GC/MS analysis of urine in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
Shinka T; Kuhara T; Inoue Y; Matsumoto M; Matsumoto I; Nakamura H; Irimichi H; Hasumi K; Endo A
Acta Paediatr Jpn; 1992 Apr; 34(2):157-65. PubMed ID: 1377861
[TBL] [Abstract][Full Text] [Related]
17. Evaluation of cysteine 266 of human 3-hydroxy-3-methylglutaryl-CoA lyase as a catalytic residue.
Roberts JR; Narasimhan C; Miziorko HM
J Biol Chem; 1995 Jul; 270(29):17311-6. PubMed ID: 7615532
[TBL] [Abstract][Full Text] [Related]
18. The Pseudomonas aeruginosa liuE gene encodes the 3-hydroxy-3-methylglutaryl coenzyme A lyase, involved in leucine and acyclic terpene catabolism.
Chávez-Avilés M; Díaz-Pérez AL; Reyes-de la Cruz H; Campos-García J
FEMS Microbiol Lett; 2009 Jul; 296(1):117-23. PubMed ID: 19459965
[TBL] [Abstract][Full Text] [Related]
19. A nonsense mutation in the 3-hydroxy-3-methylglutaryl-CoA lyase gene produces exon skipping in two patients of different origin with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
Pié J; Casals N; Casale CH; Buesa C; Mascaró C; Barceló A; Rolland MO; Zabot T; Haro D; Eyskens F; Divry P; Hegardt FG
Biochem J; 1997 Apr; 323 ( Pt 2)(Pt 2):329-35. PubMed ID: 9163320
[TBL] [Abstract][Full Text] [Related]
20. Modeling of a mutation responsible for human 3-hydroxy-3-methylglutaryl-CoA lyase deficiency implicates histidine 233 as an active site residue.
Roberts JR; Mitchell GA; Miziorko HM
J Biol Chem; 1996 Oct; 271(40):24604-9. PubMed ID: 8798725
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]