116 related articles for article (PubMed ID: 7958339)
1. The mitochondrial long-chain trifunctional enzyme: 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase and 3-oxoacyl-CoA thiolase.
Middleton B
Biochem Soc Trans; 1994 May; 22(2):427-31. PubMed ID: 7958339
[No Abstract] [Full Text] [Related]
2. The mitochondrial trifunctional protein: centre of a beta-oxidation metabolon?
Eaton S; Bursby T; Middleton B; Pourfarzam M; Mills K; Johnson AW; Bartlett K
Biochem Soc Trans; 2000 Feb; 28(2):177-82. PubMed ID: 10816122
[TBL] [Abstract][Full Text] [Related]
3. A new inhibitor of mitochondrial fatty acid oxidation.
Hashimoto T; Shindo Y; Souri M; Baldwin GS
J Biochem; 1996 Jun; 119(6):1196-201. PubMed ID: 8827458
[TBL] [Abstract][Full Text] [Related]
4. A new type of a multifunctional beta-oxidation enzyme in euglena.
Winkler U; Säftel W; Stabenau H
Plant Physiol; 2003 Feb; 131(2):753-62. PubMed ID: 12586899
[TBL] [Abstract][Full Text] [Related]
5. Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein.
Kamijo T; Aoyama T; Komiyama A; Hashimoto T
Biochem Biophys Res Commun; 1994 Mar; 199(2):818-25. PubMed ID: 8135828
[TBL] [Abstract][Full Text] [Related]
6. Reconstitution, morphology and crystallization of a fatty acid beta-oxidation multienzyme complex from Pseudomonas fragi.
Ishikawa M; Mikami Y; Usukura J; Iwasaki H; Shinagawa H; Morikawa K
Biochem J; 1997 Dec; 328 ( Pt 3)(Pt 3):815-20. PubMed ID: 9396725
[TBL] [Abstract][Full Text] [Related]
7. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: high frequency of the G1528C mutation with no apparent correlation with the clinical phenotype.
Ijlst L; Uskikubo S; Kamijo T; Hashimoto T; Ruiter JP; de Klerk JB; Wanders RJ
J Inherit Metab Dis; 1995; 18(2):241-4. PubMed ID: 7564258
[No Abstract] [Full Text] [Related]
8. Fatty acid beta-oxidation in glyoxysomes. Characterization of a new tetrafunctional protein (MFP III).
Gühnemann-Schäfer K; Kindl H
Biochim Biophys Acta; 1995 May; 1256(2):181-6. PubMed ID: 7766696
[TBL] [Abstract][Full Text] [Related]
9. Combined enzyme defect of mitochondrial fatty acid oxidation.
Jackson S; Kler RS; Bartlett K; Briggs H; Bindoff LA; Pourfarzam M; Gardner-Medwin D; Turnbull DM
J Clin Invest; 1992 Oct; 90(4):1219-25. PubMed ID: 1401059
[TBL] [Abstract][Full Text] [Related]
10. Isolated mitochondrial long-chain ketoacyl-CoA thiolase deficiency resulting from mutations in the HADHB gene.
Das AM; Illsinger S; Lücke T; Hartmann H; Ruiter JP; Steuerwald U; Waterham HR; Duran M; Wanders RJ
Clin Chem; 2006 Mar; 52(3):530-4. PubMed ID: 16423905
[TBL] [Abstract][Full Text] [Related]
11. Human trifunctional protein deficiency: a new disorder of mitochondrial fatty acid beta-oxidation.
Wanders RJ; IJlst L; Poggi F; Bonnefont JP; Munnich A; Brivet M; Rabier D; Saudubray JM
Biochem Biophys Res Commun; 1992 Nov; 188(3):1139-45. PubMed ID: 1445348
[TBL] [Abstract][Full Text] [Related]
12. Glutamate-119 of the large alpha-subunit is the catalytic base in the hydration of 2-trans-enoyl-coenzyme A catalyzed by the multienzyme complex of fatty acid oxidation from Escherichia coli.
He XY; Yang SY
Biochemistry; 1997 Sep; 36(36):11044-9. PubMed ID: 9283097
[TBL] [Abstract][Full Text] [Related]
13. Mitochondrial trifunctional protein deficiency. Catalytic heterogeneity of the mutant enzyme in two patients.
Kamijo T; Wanders RJ; Saudubray JM; Aoyama T; Komiyama A; Hashimoto T
J Clin Invest; 1994 Apr; 93(4):1740-7. PubMed ID: 8163672
[TBL] [Abstract][Full Text] [Related]
14. Fluorescence in situ hybridization mapping of the alpha and beta subunits (HADHA and HADHB) of human mitochondrial fatty acid beta-oxidation multienzyme complex to 2p23 and their evolution.
Aoyama T; Wakui K; Orii KE; Hashimoto T; Fukushima Y
Cytogenet Cell Genet; 1997; 79(3-4):221-4. PubMed ID: 9605857
[TBL] [Abstract][Full Text] [Related]
15. Comparison of metabolic fluxes of cis-5-enoyl-CoA and saturated acyl-CoA through the beta-oxidation pathway.
Tserng KY; Chen LS; Jin SJ
Biochem J; 1995 Apr; 307 ( Pt 1)(Pt 1):23-8. PubMed ID: 7717980
[TBL] [Abstract][Full Text] [Related]
16. Genomic and mutational analysis of the mitochondrial trifunctional protein beta-subunit (HADHB) gene in patients with trifunctional protein deficiency.
Orii KE; Aoyama T; Wakui K; Fukushima Y; Miyajima H; Yamaguchi S; Orii T; Kondo N; Hashimoto T
Hum Mol Genet; 1997 Aug; 6(8):1215-24. PubMed ID: 9259266
[TBL] [Abstract][Full Text] [Related]
17. Comparison of sequences of the 78 kDa gastrin-binding protein and some enzymes involved in fatty acid oxidation.
Baldwin GS
Comp Biochem Physiol B; 1993 Jan; 104(1):55-61. PubMed ID: 8448993
[TBL] [Abstract][Full Text] [Related]
18. Molecular basis of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of the major disease-causing mutation in the alpha-subunit of the mitochondrial trifunctional protein.
IJlst L; Wanders RJ; Ushikubo S; Kamijo T; Hashimoto T
Biochim Biophys Acta; 1994 Dec; 1215(3):347-50. PubMed ID: 7811722
[TBL] [Abstract][Full Text] [Related]
19. Recombinant 2-enoyl-CoA hydratase derived from rat peroxisomal multifunctional enzyme 2: role of the hydratase reaction in bile acid synthesis.
Qin YM; Haapalainen AM; Conry D; Cuebas DA; Hiltunen JK; Novikov DK
Biochem J; 1997 Dec; 328 ( Pt 2)(Pt 2):377-82. PubMed ID: 9371691
[TBL] [Abstract][Full Text] [Related]
20. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women.
Ibdah JA; Bennett MJ; Rinaldo P; Zhao Y; Gibson B; Sims HF; Strauss AW
N Engl J Med; 1999 Jun; 340(22):1723-31. PubMed ID: 10352164
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]