288 related articles for article (PubMed ID: 24158852)
1. ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies.
Nouws J; Te Brinke H; Nijtmans LG; Houten SM
Hum Mol Genet; 2014 Mar; 23(5):1311-9. PubMed ID: 24158852
[TBL] [Abstract][Full Text] [Related]
2. Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex I.
Nouws J; Nijtmans L; Houten SM; van den Brand M; Huynen M; Venselaar H; Hoefs S; Gloerich J; Kronick J; Hutchin T; Willems P; Rodenburg R; Wanders R; van den Heuvel L; Smeitink J; Vogel RO
Cell Metab; 2010 Sep; 12(3):283-94. PubMed ID: 20816094
[TBL] [Abstract][Full Text] [Related]
3. Tissue-specific strategies of the very-long chain acyl-CoA dehydrogenase-deficient (VLCAD-/-) mouse to compensate a defective fatty acid β-oxidation.
Tucci S; Herebian D; Sturm M; Seibt A; Spiekerkoetter U
PLoS One; 2012; 7(9):e45429. PubMed ID: 23024820
[TBL] [Abstract][Full Text] [Related]
4. Complex I assembly function and fatty acid oxidation enzyme activity of ACAD9 both contribute to disease severity in ACAD9 deficiency.
Schiff M; Haberberger B; Xia C; Mohsen AW; Goetzman ES; Wang Y; Uppala R; Zhang Y; Karunanidhi A; Prabhu D; Alharbi H; Prochownik EV; Haack T; Häberle J; Munnich A; Rötig A; Taylor RW; Nicholls RD; Kim JJ; Prokisch H; Vockley J
Hum Mol Genet; 2015 Jun; 24(11):3238-47. PubMed ID: 25721401
[TBL] [Abstract][Full Text] [Related]
5. Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship.
Gregersen N; Andresen BS; Corydon MJ; Corydon TJ; Olsen RK; Bolund L; Bross P
Hum Mutat; 2001 Sep; 18(3):169-89. PubMed ID: 11524729
[TBL] [Abstract][Full Text] [Related]
6. Effect of heat stress and bezafibrate on mitochondrial beta-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay.
Li H; Fukuda S; Hasegawa Y; Kobayashi H; Purevsuren J; Mushimoto Y; Yamaguchi S
Brain Dev; 2010 May; 32(5):362-70. PubMed ID: 19589653
[TBL] [Abstract][Full Text] [Related]
7. Analysis of carnitine esters by radio-high performance liquid chromatography in cultured skin fibroblasts from patients with mitochondrial fatty acid oxidation disorders.
Schmidt-Sommerfeld E; Bobrowski PJ; Penn D; Rhead WJ; Wanders RJ; Bennett MJ
Pediatr Res; 1998 Aug; 44(2):210-4. PubMed ID: 9702916
[TBL] [Abstract][Full Text] [Related]
8. De novo fatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even medium-chain fatty acids.
Tucci S; Behringer S; Spiekerkoetter U
FEBS J; 2015 Nov; 282(21):4242-53. PubMed ID: 26284828
[TBL] [Abstract][Full Text] [Related]
9. Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders.
Djouadi F; Aubey F; Schlemmer D; Ruiter JP; Wanders RJ; Strauss AW; Bastin J
Hum Mol Genet; 2005 Sep; 14(18):2695-703. PubMed ID: 16115821
[TBL] [Abstract][Full Text] [Related]
10. Food withdrawal lowers energy expenditure and induces inactivity in long-chain fatty acid oxidation-deficient mouse models.
Diekman EF; van Weeghel M; Wanders RJ; Visser G; Houten SM
FASEB J; 2014 Jul; 28(7):2891-900. PubMed ID: 24648546
[TBL] [Abstract][Full Text] [Related]
11. Long-chain fatty acid oxidation during early human development.
Oey NA; den Boer ME; Wijburg FA; Vekemans M; Augé J; Steiner C; Wanders RJ; Waterham HR; Ruiter JP; Attié-Bitach T
Pediatr Res; 2005 Jun; 57(6):755-9. PubMed ID: 15845636
[TBL] [Abstract][Full Text] [Related]
12. Evaluation of Metabolic Defects in Fatty Acid Oxidation Using Peripheral Blood Mononuclear Cells Loaded with Deuterium-Labeled Fatty Acids.
Yuasa M; Hata I; Sugihara K; Isozaki Y; Ohshima Y; Hara K; Tajima G; Shigematsu Y
Dis Markers; 2019; 2019():2984747. PubMed ID: 30881520
[TBL] [Abstract][Full Text] [Related]
13. Prenatal diagnosis of mitochondrial fatty acid oxidation defects.
Nada MA; Vianey-Saban C; Roe CR; Ding JH; Mathieu M; Wappner RS; Bialer MG; McGlynn JA; Mandon G
Prenat Diagn; 1996 Feb; 16(2):117-24. PubMed ID: 8650121
[TBL] [Abstract][Full Text] [Related]
14. Neonatal multiorgan failure due to ACAD9 mutation and complex I deficiency with mitochondrial hyperplasia in liver, cardiac myocytes, skeletal muscle, and renal tubules.
Leslie N; Wang X; Peng Y; Valencia CA; Khuchua Z; Hata J; Witte D; Huang T; Bove KE
Hum Pathol; 2016 Mar; 49():27-32. PubMed ID: 26826406
[TBL] [Abstract][Full Text] [Related]
15. The effect of valinomycin in fibroblasts from patients with fatty acid oxidation disorders.
Ndukwe Erlingsson UC; Iacobazzi F; Liu A; Ardon O; Pasquali M; Longo N
Biochem Biophys Res Commun; 2013 Aug; 437(4):637-41. PubMed ID: 23867825
[TBL] [Abstract][Full Text] [Related]
16. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels.
Liebig M; Schymik I; Mueller M; Wendel U; Mayatepek E; Ruiter J; Strauss AW; Wanders RJ; Spiekerkoetter U
Pediatrics; 2006 Sep; 118(3):1065-9. PubMed ID: 16950999
[TBL] [Abstract][Full Text] [Related]
17. Heat stress deteriorates mitochondrial beta-oxidation of long-chain fatty acids in cultured fibroblasts with fatty acid beta-oxidation disorders.
Li H; Fukuda S; Hasegawa Y; Purevsuren J; Kobayashi H; Mushimoto Y; Yamaguchi S
J Chromatogr B Analyt Technol Biomed Life Sci; 2010 Jun; 878(20):1669-72. PubMed ID: 20207594
[TBL] [Abstract][Full Text] [Related]
18. Cardiac tissue citric acid cycle intermediates in exercised very long-chain acyl-CoA dehydrogenase-deficient mice fed triheptanoin or medium-chain triglyceride.
Gaston G; Gangoiti JA; Winn S; Chan B; Barshop BA; Harding CO; Gillingham MB
J Inherit Metab Dis; 2020 Nov; 43(6):1232-1242. PubMed ID: 33448436
[TBL] [Abstract][Full Text] [Related]
19. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.
Tyni T; Paetau A; Strauss AW; Middleton B; Kivelä T
Pediatr Res; 2004 Nov; 56(5):744-50. PubMed ID: 15347768
[TBL] [Abstract][Full Text] [Related]
20. In vivo stable isotope studies in three patients affected with mitochondrial fatty acid oxidation disorders: limited diagnostic use of 1-13C fatty acid breath test using bolus technique.
Jakobs C; Kneer J; Martin D; Boulloche J; Brivet M; Poll-The BT; Saudubray JM
Eur J Pediatr; 1997 Aug; 156 Suppl 1():S78-82. PubMed ID: 9266222
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
