124 related articles for article (PubMed ID: 37558007)
21. Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy.
Keeler AM; Conlon T; Walter G; Zeng H; Shaffer SA; Dungtao F; Erger K; Cossette T; Tang Q; Mueller C; Flotte TR
Mol Ther; 2012 Jun; 20(6):1131-8. PubMed ID: 22395529
[TBL] [Abstract][Full Text] [Related]
22. Diagnosis of very long chain acyl-dehydrogenase deficiency from an infant's newborn screening card.
Wood JC; Magera MJ; Rinaldo P; Seashore MR; Strauss AW; Friedman A
Pediatrics; 2001 Jul; 108(1):E19. PubMed ID: 11433098
[TBL] [Abstract][Full Text] [Related]
23. The fatty acid oxidation enzyme long-chain acyl-CoA dehydrogenase can be a source of mitochondrial hydrogen peroxide.
Zhang Y; Bharathi SS; Beck ME; Goetzman ES
Redox Biol; 2019 Sep; 26():101253. PubMed ID: 31234015
[TBL] [Abstract][Full Text] [Related]
24. Fasting-induced oxidative stress in very long chain acyl-CoA dehydrogenase-deficient mice.
Tucci S; Primassin S; Spiekerkoetter U
FEBS J; 2010 Nov; 277(22):4699-708. PubMed ID: 20883455
[TBL] [Abstract][Full Text] [Related]
25. Long-chain acyl-CoA dehydrogenase is a key enzyme in the mitochondrial beta-oxidation of unsaturated fatty acids.
Lea W; Abbas AS; Sprecher H; Vockley J; Schulz H
Biochim Biophys Acta; 2000 May; 1485(2-3):121-8. PubMed ID: 10832093
[TBL] [Abstract][Full Text] [Related]
26. Metabolite accumulation in VLCAD deficiency markedly disrupts mitochondrial bioenergetics and Ca
Cecatto C; Amaral AU; da Silva JC; Wajner A; Schimit MOV; da Silva LHR; Wajner SM; Zanatta Â; Castilho RF; Wajner M
FEBS J; 2018 Apr; 285(8):1437-1455. PubMed ID: 29476646
[TBL] [Abstract][Full Text] [Related]
27. Hepatic and muscular effects of different dietary fat content in VLCAD deficient mice.
Primassin S; Tucci S; Spiekerkoetter U
Mol Genet Metab; 2011 Dec; 104(4):546-51. PubMed ID: 21963783
[TBL] [Abstract][Full Text] [Related]
28. Recurrent ACADVL molecular findings in individuals with a positive newborn screen for very long chain acyl-coA dehydrogenase (VLCAD) deficiency in the United States.
Miller MJ; Burrage LC; Gibson JB; Strenk ME; Lose EJ; Bick DP; Elsea SH; Sutton VR; Sun Q; Graham BH; Craigen WJ; Zhang VW; Wong LJ
Mol Genet Metab; 2015 Nov; 116(3):139-45. PubMed ID: 26385305
[TBL] [Abstract][Full Text] [Related]
29. Novel ACADVL variants resulting in mitochondrial defects in long-chain acyl-CoA dehydrogenase deficiency.
Chen T; Tong F; Wu XY; Zhu L; Yi QZ; Zheng J; Yang RL; Zhao ZY; Cang XH; Shu Q; Jiang PP
J Zhejiang Univ Sci B; 2020 Nov.; 21(11):885-896. PubMed ID: 33150772
[TBL] [Abstract][Full Text] [Related]
30. Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress.
Spiekerkoetter U; Tokunaga C; Wendel U; Mayatepek E; Exil V; Duran M; Wijburg FA; Wanders RJ; Strauss AW
Eur J Clin Invest; 2004 Mar; 34(3):191-6. PubMed ID: 15025677
[TBL] [Abstract][Full Text] [Related]
31. Synthetic mRNA rescues very long-chain acyl-CoA dehydrogenase deficiency in patient fibroblasts and a murine model.
Zhao XJ; Mohsen AW; Mihalik S; Solo K; Aliu E; Shi H; Basu S; Kochersperger C; Van't Land C; Karunanidhi A; Coughlan KA; Siddiqui S; Rice LM; Hillier S; Guadagnin E; Giangrande PH; Martini PGV; Vockley J
Mol Genet Metab; 2023 Jan; 138(1):106982. PubMed ID: 36580829
[TBL] [Abstract][Full Text] [Related]
32. Strategies for correcting very long chain acyl-CoA dehydrogenase deficiency.
Tenopoulou M; Chen J; Bastin J; Bennett MJ; Ischiropoulos H; Doulias PT
J Biol Chem; 2015 Apr; 290(16):10486-94. PubMed ID: 25737446
[TBL] [Abstract][Full Text] [Related]
33. Mitochondrial long chain fatty acid beta-oxidation in man and mouse.
Chegary M; Brinke Ht; Ruiter JP; Wijburg FA; Stoll MS; Minkler PE; van Weeghel M; Schulz H; Hoppel CL; Wanders RJ; Houten SM
Biochim Biophys Acta; 2009 Aug; 1791(8):806-15. PubMed ID: 19465148
[TBL] [Abstract][Full Text] [Related]
34. Sexual dimorphism of lipid metabolism in very long-chain acyl-CoA dehydrogenase deficient (VLCAD-/-) mice in response to medium-chain triglycerides (MCT).
Tucci S; Flögel U; Spiekerkoetter U
Biochim Biophys Acta; 2015 Jul; 1852(7):1442-50. PubMed ID: 25887160
[TBL] [Abstract][Full Text] [Related]
35. Clinical and biochemical outcome of patients with very long-chain acyl-CoA dehydrogenase deficiency.
Rovelli V; Manzoni F; Viau K; Pasquali M; Longo N
Mol Genet Metab; 2019 May; 127(1):64-73. PubMed ID: 31031081
[TBL] [Abstract][Full Text] [Related]
36. Medium branched chain fatty acids improve the profile of tricarboxylic acid cycle intermediates in mitochondrial fatty acid β-oxidation deficient cells: A comparative study.
Karunanidhi A; Van't Land C; Rajasundaram D; Grings M; Vockley J; Mohsen AW
J Inherit Metab Dis; 2022 May; 45(3):541-556. PubMed ID: 35076099
[TBL] [Abstract][Full Text] [Related]
37. 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]
38. Long-term dietary effects on substrate selection and muscle fiber type in very-long-chain acyl-CoA dehydrogenase deficient (VLCAD(-/-)) mice.
Tucci S; Pearson S; Herebian D; Spiekerkoetter U
Biochim Biophys Acta; 2013 Apr; 1832(4):509-16. PubMed ID: 23313579
[TBL] [Abstract][Full Text] [Related]
39. Management and diagnosis of mitochondrial fatty acid oxidation disorders: focus on very-long-chain acyl-CoA dehydrogenase deficiency.
Yamada K; Taketani T
J Hum Genet; 2019 Feb; 64(2):73-85. PubMed ID: 30401918
[TBL] [Abstract][Full Text] [Related]
40. Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial beta-oxidation defects.
Onkenhout W; Venizelos V; Scholte HR; De Klerk JB; Poorthuis BJ
J Inherit Metab Dis; 2001 Jun; 24(3):337-44. PubMed ID: 11486898
[TBL] [Abstract][Full Text] [Related]
[Previous] [Next] [New Search]