429 related articles for article (PubMed ID: 16617370)
1. [Study of the inborn errors of mitochondrial fatty acid beta-oxidation deficiency].
Zhu JM; Yang Z
Beijing Da Xue Xue Bao Yi Xue Ban; 2006 Apr; 38(2):214-7. PubMed ID: 16617370
[TBL] [Abstract][Full Text] [Related]
2. 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]
3. 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]
4. 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]
5. New genetic defects in mitochondrial fatty acid oxidation and carnitine deficiency.
Stanley CA
Adv Pediatr; 1987; 34():59-88. PubMed ID: 3318304
[TBL] [Abstract][Full Text] [Related]
6. 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]
7. Prevalent mutations in fatty acid oxidation disorders: diagnostic considerations.
Gregersen N; Andresen BS; Bross P
Eur J Pediatr; 2000 Dec; 159 Suppl 3():S213-8. PubMed ID: 11216903
[TBL] [Abstract][Full Text] [Related]
8. Diagnosis of mitochondrial fatty acid oxidation defects.
Duran M; Bruinvis L; Ketting D; Dorland L
Padiatr Padol; 1993; 28(1):19-25. PubMed ID: 8446424
[TBL] [Abstract][Full Text] [Related]
9. 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]
10. Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders.
van Eunen K; Volker-Touw CM; Gerding A; Bleeker A; Wolters JC; van Rijt WJ; Martines AM; Niezen-Koning KE; Heiner RM; Permentier H; Groen AK; Reijngoud DJ; Derks TG; Bakker BM
BMC Biol; 2016 Dec; 14(1):107. PubMed ID: 27927213
[TBL] [Abstract][Full Text] [Related]
11. Genetic and cellular modifiers of oxidative stress: what can we learn from fatty acid oxidation defects?
Olsen RK; Cornelius N; Gregersen N
Mol Genet Metab; 2013; 110 Suppl():S31-9. PubMed ID: 24206932
[TBL] [Abstract][Full Text] [Related]
12. Oxidation of fatty acids in cultured fibroblasts: a model system for the detection and study of defects in oxidation.
Saudubray JM; Coudé FX; Demaugre F; Johnson C; Gibson KM; Nyhan WL
Pediatr Res; 1982 Oct; 16(10):877-81. PubMed ID: 7145511
[TBL] [Abstract][Full Text] [Related]
13. 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]
14. Mammalian mitochondrial beta-oxidation.
Eaton S; Bartlett K; Pourfarzam M
Biochem J; 1996 Dec; 320 ( Pt 2)(Pt 2):345-57. PubMed ID: 8973539
[TBL] [Abstract][Full Text] [Related]
15. Heptanoic and medium branched-chain fatty acids as anaplerotic treatment for medium chain acyl-CoA dehydrogenase deficiency.
Karunanidhi A; Basu S; Zhao XJ; D'Annibale O; Van't Land C; Vockley J; Mohsen AW
Mol Genet Metab; 2023 Nov; 140(3):107689. PubMed ID: 37660571
[TBL] [Abstract][Full Text] [Related]
16. 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]
17. Dicarboxylic aciduria: deficient [1-14C]octanoate oxidation and medium-chain acyl-CoA dehydrogenase in fibroblasts.
Rhead WJ; Amendt BA; Fritchman KS; Felts SJ
Science; 1983 Jul; 221(4605):73-5. PubMed ID: 6857268
[TBL] [Abstract][Full Text] [Related]
18. 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]
19. A rare disease-associated mutation in the medium-chain acyl-CoA dehydrogenase (MCAD) gene changes a conserved arginine, previously shown to be functionally essential in short-chain acyl-CoA dehydrogenase (SCAD).
Andresen BS; Bross P; Jensen TG; Winter V; Knudsen I; Kølvraa S; Jensen UB; Bolund L; Duran M; Kim JJ
Am J Hum Genet; 1993 Sep; 53(3):730-9. PubMed ID: 8102510
[TBL] [Abstract][Full Text] [Related]
20. Accumulation of 3-hydroxy-fatty acids in the culture medium of long-chain L-3-hydroxyacyl CoA dehydrogenase (LCHAD) and mitochondrial trifunctional protein-deficient skin fibroblasts: implications for medium chain triglyceride dietary treatment of LCHAD deficiency.
Jones PM; Butt Y; Bennett MJ
Pediatr Res; 2003 May; 53(5):783-7. PubMed ID: 12621125
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
