184 related articles for article (PubMed ID: 3326738)
1. Molecular basis of isovaleric acidemia and medium-chain acyl-CoA dehydrogenase deficiency.
Tanaka K; Ikeda Y; Matsubara Y; Hyman DB
Enzyme; 1987; 38(1-4):91-107. PubMed ID: 3326738
[TBL] [Abstract][Full Text] [Related]
2. Molecular basis of isovaleric acidemia and the study of the acyl-CoA dehydrogenase family.
Tanaka K; Ikeda Y; Matsubara Y; Hyman D
Adv Neurol; 1988; 48():107-31. PubMed ID: 3275439
[No Abstract] [Full Text] [Related]
3. Nucleotide sequence of messenger RNA encoding human isovaleryl-coenzyme A dehydrogenase and its expression in isovaleric acidemia fibroblasts.
Matsubara Y; Ito M; Glassberg R; Satyabhama S; Ikeda Y; Tanaka K
J Clin Invest; 1990 Apr; 85(4):1058-64. PubMed ID: 2318964
[TBL] [Abstract][Full Text] [Related]
4. Molecular characterization of four different classes of mutations in the isovaleryl-CoA dehydrogenase gene responsible for isovaleric acidemia.
Vockley J; Parimoo B; Tanaka K
Am J Hum Genet; 1991 Jul; 49(1):147-57. PubMed ID: 2063866
[TBL] [Abstract][Full Text] [Related]
5. Isovaleryl-CoA dehydrogenase activity in isovaleric acidemia fibroblasts using an improved tritium release assay.
Hyman DB; Tanaka K
Pediatr Res; 1986 Jan; 20(1):59-61. PubMed ID: 3753755
[TBL] [Abstract][Full Text] [Related]
6. [Isovaleric acidemia].
Ikeda Y; Tanaka K
Tanpakushitsu Kakusan Koso; 1988 Apr; 33(5):554-9. PubMed ID: 3270862
[No Abstract] [Full Text] [Related]
7. Molecular cloning and nucleotide sequence of cDNAs encoding the precursors of rat long chain acyl-coenzyme A, short chain acyl-coenzyme A, and isovaleryl-coenzyme A dehydrogenases. Sequence homology of four enzymes of the acyl-CoA dehydrogenase family.
Matsubara Y; Indo Y; Naito E; Ozasa H; Glassberg R; Vockley J; Ikeda Y; Kraus J; Tanaka K
J Biol Chem; 1989 Sep; 264(27):16321-31. PubMed ID: 2777793
[TBL] [Abstract][Full Text] [Related]
8. The variant human isovaleryl-CoA dehydrogenase gene responsible for type II isovaleric acidemia determines an RNA splicing error, leading to the deletion of the entire second coding exon and the production of a truncated precursor protein that interacts poorly with mitochondrial import receptors.
Vockley J; Nagao M; Parimoo B; Tanaka K
J Biol Chem; 1992 Feb; 267(4):2494-501. PubMed ID: 1310317
[TBL] [Abstract][Full Text] [Related]
9. Exon skipping in IVD RNA processing in isovaleric acidemia caused by point mutations in the coding region of the IVD gene.
Vockley J; Rogan PK; Anderson BD; Willard J; Seelan RS; Smith DI; Liu W
Am J Hum Genet; 2000 Feb; 66(2):356-67. PubMed ID: 10677295
[TBL] [Abstract][Full Text] [Related]
10. Neonatal isovaleric acidemia associated with hyperammonemia.
Yoshino M; Yoshida I; Yamashita F; Mori M; Uchiyama C; Tatibana M
Adv Exp Med Biol; 1982; 153():141-6. PubMed ID: 6897695
[No Abstract] [Full Text] [Related]
11. Mitochondrial import and processing of wild type and type III mutant isovaleryl-CoA dehydrogenase.
Volchenboum SL; Vockley J
J Biol Chem; 2000 Mar; 275(11):7958-63. PubMed ID: 10713113
[TBL] [Abstract][Full Text] [Related]
12. [Isovaleric acidemia].
Yoshida I
Ryoikibetsu Shokogun Shirizu; 1998; (18 Pt 1):280-3. PubMed ID: 9590047
[No Abstract] [Full Text] [Related]
13. Structure of human isovaleryl-CoA dehydrogenase at 2.6 A resolution: structural basis for substrate specificity,
Tiffany KA; Roberts DL; Wang M; Paschke R; Mohsen AW; Vockley J; Kim JJ
Biochemistry; 1997 Jul; 36(28):8455-64. PubMed ID: 9214289
[TBL] [Abstract][Full Text] [Related]
14. The treatment of isovaleric acidemia with glycine supplement.
Naglak M; Salvo R; Madsen K; Dembure P; Elsas L
Pediatr Res; 1988 Jul; 24(1):9-13. PubMed ID: 3137519
[TBL] [Abstract][Full Text] [Related]
15. Acute and chronic-intermittent isovaleric acidemia: diagnosis and glycine therapy.
Elsas LJ; Naglak M
Acta Paediatr Jpn; 1988 Aug; 30(4):442-51. PubMed ID: 3150234
[No Abstract] [Full Text] [Related]
16. A Scandinavian case of isovaleric acidaemia.
Gerdes AM; Gregersen N; Lúdvigsson P; Güttler F
J Inherit Metab Dis; 1988; 11(2):219-20. PubMed ID: 3139936
[No Abstract] [Full Text] [Related]
17. Identification of Caenorhabditis elegans isovaleryl-CoA dehydrogenase and structural comparison with other acyl-CoA dehydrogenases.
Mohsen AW; Navarette B; Vockley J
Mol Genet Metab; 2001 Jun; 73(2):126-37. PubMed ID: 11386848
[TBL] [Abstract][Full Text] [Related]
18. Cloning of a gene for an acyl-CoA dehydrogenase from Pisum sativum L. and purification and characterization of its product as an isovaleryl-CoA dehydrogenase.
Reinard T; Janke V; Willard J; Buck F; Jacobsen HJ; Vockley J
J Biol Chem; 2000 Oct; 275(43):33738-43. PubMed ID: 10913142
[TBL] [Abstract][Full Text] [Related]
19. L-carnitine therapy in isovaleric acidemia.
Roe CR; Millington DS; Maltby DA; Kahler SG; Bohan TP
J Clin Invest; 1984 Dec; 74(6):2290-5. PubMed ID: 6549017
[TBL] [Abstract][Full Text] [Related]
20. Catalytic defect of medium-chain acyl-coenzyme A dehydrogenase deficiency. Lack of both cofactor responsiveness and biochemical heterogeneity in eight patients.
Amendt BA; Rhead WJ
J Clin Invest; 1985 Sep; 76(3):963-9. PubMed ID: 3840178
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
