234 related articles for article (PubMed ID: 27371118)
1. Disturbance of mitochondrial functions provoked by the major long-chain 3-hydroxylated fatty acids accumulating in MTP and LCHAD deficiencies in skeletal muscle.
Cecatto C; Godoy KDS; da Silva JC; Amaral AU; Wajner M
Toxicol In Vitro; 2016 Oct; 36():1-9. PubMed ID: 27371118
[TBL] [Abstract][Full Text] [Related]
2. Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart--mitochondrial permeability transition pore opening as a potential contributing pathomechanism of cardiac alterations in these disorders.
Cecatto C; Hickmann FH; Rodrigues MD; Amaral AU; Wajner M
FEBS J; 2015 Dec; 282(24):4714-26. PubMed ID: 26408230
[TBL] [Abstract][Full Text] [Related]
3. Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency.
Hickmann FH; Cecatto C; Kleemann D; Monteiro WO; Castilho RF; Amaral AU; Wajner M
Biochim Biophys Acta; 2015; 1847(6-7):620-8. PubMed ID: 25868874
[TBL] [Abstract][Full Text] [Related]
4. Mitochondrial bioenergetics deregulation caused by long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies in rat brain: a possible role of mPTP opening as a pathomechanism in these disorders?
Tonin AM; Amaral AU; Busanello EN; Gasparotto J; Gelain DP; Gregersen N; Wajner M
Biochim Biophys Acta; 2014 Sep; 1842(9):1658-67. PubMed ID: 24946182
[TBL] [Abstract][Full Text] [Related]
5. Long-chain 3-hydroxy fatty acids accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial trifunctional protein deficiencies uncouple oxidative phosphorylation in heart mitochondria.
Tonin AM; Amaral AU; Busanello EN; Grings M; Castilho RF; Wajner M
J Bioenerg Biomembr; 2013 Feb; 45(1-2):47-57. PubMed ID: 23065309
[TBL] [Abstract][Full Text] [Related]
6. Disturbance of mitochondrial energy homeostasis caused by the metabolites accumulating in LCHAD and MTP deficiencies in rat brain.
Tonin AM; Ferreira GC; Grings M; Viegas CM; Busanello EN; Amaral AU; Zanatta A; Schuck PF; Wajner M
Life Sci; 2010 May; 86(21-22):825-31. PubMed ID: 20399795
[TBL] [Abstract][Full Text] [Related]
7. Impairment of mitochondrial bioenergetics and permeability transition induction caused by major long-chain fatty acids accumulating in VLCAD deficiency in skeletal muscle as potential pathomechanisms of myopathy.
Cecatto C; Amaral AU; Roginski AC; Castilho RF; Wajner M
Toxicol In Vitro; 2020 Feb; 62():104665. PubMed ID: 31629068
[TBL] [Abstract][Full Text] [Related]
8. Long-chain 3-hydroxy fatty acids accumulating in LCHAD and MTP deficiencies induce oxidative stress in rat brain.
Tonin AM; Grings M; Busanello EN; Moura AP; Ferreira GC; Viegas CM; Fernandes CG; Schuck PF; Wajner M
Neurochem Int; 2010 Jul; 56(8):930-6. PubMed ID: 20381565
[TBL] [Abstract][Full Text] [Related]
9. Fatal pitfalls in newborn screening for mitochondrial trifunctional protein (MTP)/long-chain 3-Hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency.
Lotz-Havla AS; Röschinger W; Schiergens K; Singer K; Karall D; Konstantopoulou V; Wortmann SB; Maier EM
Orphanet J Rare Dis; 2018 Jul; 13(1):122. PubMed ID: 30029694
[TBL] [Abstract][Full Text] [Related]
10. 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]
11. Pharmacological inhibition of carnitine palmitoyltransferase 1 restores mitochondrial oxidative phosphorylation in human trifunctional protein deficient fibroblasts.
Lefort B; Gouache E; Acquaviva C; Tardieu M; Benoist JF; Dumas JF; Servais S; Chevalier S; Vianey-Saban C; Labarthe F
Biochim Biophys Acta Mol Basis Dis; 2017 Jun; 1863(6):1292-1299. PubMed ID: 28392417
[TBL] [Abstract][Full Text] [Related]
12. cis-4-Decenoic and decanoic acids impair mitochondrial energy, redox and Ca(2+) homeostasis and induce mitochondrial permeability transition pore opening in rat brain and liver: Possible implications for the pathogenesis of MCAD deficiency.
Amaral AU; Cecatto C; da Silva JC; Wajner A; Godoy KDS; Ribeiro RT; Wajner M
Biochim Biophys Acta; 2016 Sep; 1857(9):1363-1372. PubMed ID: 27240720
[TBL] [Abstract][Full Text] [Related]
13. Neurological outcome in long-chain hydroxy fatty acid oxidation disorders.
Mütze U; Ottenberger A; Gleich F; Maier EM; Lindner M; Husain RA; Palm K; Beblo S; Freisinger P; Santer R; Thimm E; Vom Dahl S; Weinhold N; Grohmann-Held K; Haase C; Hennermann JB; Hörbe-Blindt A; Kamrath C; Marquardt I; Marquardt T; Behne R; Haas D; Spiekerkoetter U; Hoffmann GF; Garbade SF; Grünert SC; Kölker S
Ann Clin Transl Neurol; 2024 Apr; 11(4):883-898. PubMed ID: 38263760
[TBL] [Abstract][Full Text] [Related]
14. Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficiency: a systematic review.
Fraser H; Geppert J; Johnson R; Johnson S; Connock M; Clarke A; Taylor-Phillips S; Stinton C
Orphanet J Rare Dis; 2019 Nov; 14(1):258. PubMed ID: 31730477
[TBL] [Abstract][Full Text] [Related]
15. Triheptanoin versus trioctanoin for long-chain fatty acid oxidation disorders: a double blinded, randomized controlled trial.
Gillingham MB; Heitner SB; Martin J; Rose S; Goldstein A; El-Gharbawy AH; Deward S; Lasarev MR; Pollaro J; DeLany JP; Burchill LJ; Goodpaster B; Shoemaker J; Matern D; Harding CO; Vockley J
J Inherit Metab Dis; 2017 Nov; 40(6):831-843. PubMed ID: 28871440
[TBL] [Abstract][Full Text] [Related]
16. Thermo-sensitive mitochondrial trifunctional protein deficiency presenting with episodic myopathy.
Schwantje M; Ebberink MS; Doolaard M; Ruiter JPN; Fuchs SA; Darin N; Hedberg-Oldfors C; Régal L; Donker Kaat L; Huidekoper HH; Olpin S; Cole D; Moat SJ; Visser G; Ferdinandusse S
J Inherit Metab Dis; 2022 Jul; 45(4):819-831. PubMed ID: 35403730
[TBL] [Abstract][Full Text] [Related]
17. Impact of newborn screening for fatty acid oxidation disorders on neurological outcome: A Belgian retrospective and multicentric study.
Everard E; Laeremans H; Boemer F; Marie S; Vincent MF; Dewulf JP; Debray FG; De Laet C; Nassogne MC
Eur J Paediatr Neurol; 2024 Mar; 49():60-65. PubMed ID: 38377647
[TBL] [Abstract][Full Text] [Related]
18. Child neurology: Recurrent rhabdomyolysis due to a fatty acid oxidation disorder.
Terrone G; Ruoppolo M; Brunetti-Pierri N; Cozzolino C; Scolamiero E; Parenti G; Romano A; Andria G; Salvatore F; Frisso G
Neurology; 2014 Jan; 82(1):e1-4. PubMed ID: 24379101
[No Abstract] [Full Text] [Related]
19. Evidence that the major metabolites accumulating in medium-chain acyl-CoA dehydrogenase deficiency disturb mitochondrial energy homeostasis in rat brain.
Schuck PF; Ferreira Gda C; Tonin AM; Viegas CM; Busanello EN; Moura AP; Zanatta A; Klamt F; Wajner M
Brain Res; 2009 Nov; 1296():117-26. PubMed ID: 19703432
[TBL] [Abstract][Full Text] [Related]
20. Genetic, biochemical, and clinical spectrum of patients with mitochondrial trifunctional protein deficiency identified after the introduction of newborn screening in the Netherlands.
Schwantje M; Fuchs SA; de Boer L; Bosch AM; Cuppen I; Dekkers E; Derks TGJ; Ferdinandusse S; Ijlst L; Houtkooper RH; Maase R; van der Pol WL; de Vries MC; Verschoof-Puite RK; Wanders RJA; Williams M; Wijburg F; Visser G
J Inherit Metab Dis; 2022 Jul; 45(4):804-818. PubMed ID: 35383965
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
