Schlemmer D - Search Results

1

Monolysocardiolipins accumulate in Barth syndrome but do not lead to enhanced apoptosis.

Valianpour F, Mitsakos V, Schlemmer D, Towbin JA, Taylor JM, Ekert PG, Thorburn DR, Munnich A, Wanders RJ, Barth PG, Vaz FM. Valianpour F, et al. Among authors: schlemmer d. J Lipid Res. 2005 Jun;46(6):1182-95. doi: 10.1194/jlr.M500056-JLR200. Epub 2005 Apr 1. J Lipid Res. 2005. PMID: 15805542 Free article.

2

Mitochondrial trifunctional protein deficiency in human cultured fibroblasts: effects of bezafibrate.

Djouadi F, Habarou F, Le Bachelier C, Ferdinandusse S, Schlemmer D, Benoist JF, Boutron A, Andresen BS, Visser G, de Lonlay P, Olpin S, Fukao T, Yamaguchi S, Strauss AW, Wanders RJ, Bastin J. Djouadi F, et al. Among authors: schlemmer d. J Inherit Metab Dis. 2016 Jan;39(1):47-58. doi: 10.1007/s10545-015-9871-3. Epub 2015 Jun 25. J Inherit Metab Dis. 2016. PMID: 26109258 Free article.

3

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. Djouadi F, et al. Among authors: schlemmer d. Hum Mol Genet. 2005 Sep 15;14(18):2695-703. doi: 10.1093/hmg/ddi303. Epub 2005 Aug 22. Hum Mol Genet. 2005. PMID: 16115821

4

Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs?

Djouadi F, Aubey F, Schlemmer D, Gobin S, Laforet P, Wanders RJ, Strauss AW, Bonnefont JP, Bastin J. Djouadi F, et al. Among authors: schlemmer d. J Inherit Metab Dis. 2006 Apr-Jun;29(2-3):341-2. doi: 10.1007/s10545-006-0266-3. J Inherit Metab Dis. 2006. PMID: 16763897

5

Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders.

Mollet J, Giurgea I, Schlemmer D, Dallner G, Chretien D, Delahodde A, Bacq D, de Lonlay P, Munnich A, Rötig A. Mollet J, et al. Among authors: schlemmer d. J Clin Invest. 2007 Mar;117(3):765-72. doi: 10.1172/JCI29089. J Clin Invest. 2007. PMID: 17332895 Free PMC article.

6

CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures.

Mollet J, Delahodde A, Serre V, Chretien D, Schlemmer D, Lombes A, Boddaert N, Desguerre I, de Lonlay P, de Baulny HO, Munnich A, Rötig A. Mollet J, et al. Among authors: schlemmer d. Am J Hum Genet. 2008 Mar;82(3):623-30. doi: 10.1016/j.ajhg.2007.12.022. Am J Hum Genet. 2008. PMID: 18319072 Free PMC article.

7

A new AMPK activator, GSK773, corrects fatty acid oxidation and differentiation defect in CPT2-deficient myotubes.

Boufroura FZ, Le Bachelier C, Tomkiewicz-Raulet C, Schlemmer D, Benoist JF, Grondin P, Lamotte Y, Mirguet O, Mouillet-Richard S, Bastin J, Djouadi F. Boufroura FZ, et al. Among authors: schlemmer d. Hum Mol Genet. 2018 Oct 1;27(19):3417-3433. doi: 10.1093/hmg/ddy254. Hum Mol Genet. 2018. PMID: 30007356 Free article.

8

Peroxisome proliferator activated receptor delta (PPARdelta) agonist but not PPARalpha corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells.

Djouadi F, Aubey F, Schlemmer D, Bastin J. Djouadi F, et al. Among authors: schlemmer d. J Clin Endocrinol Metab. 2005 Mar;90(3):1791-7. doi: 10.1210/jc.2004-1936. Epub 2004 Dec 21. J Clin Endocrinol Metab. 2005. PMID: 15613406

9

Stilbenes and resveratrol metabolites improve mitochondrial fatty acid oxidation defects in human fibroblasts.

Aires V, Delmas D, Le Bachelier C, Latruffe N, Schlemmer D, Benoist JF, Djouadi F, Bastin J. Aires V, et al. Among authors: schlemmer d. Orphanet J Rare Dis. 2014 Jun 5;9:79. doi: 10.1186/1750-1172-9-79. Orphanet J Rare Dis. 2014. PMID: 24898617 Free PMC article.

10

Quinone analogues regulate mitochondrial substrate competitive oxidation.

Brière JJ, Schlemmer D, Chretien D, Rustin P. Brière JJ, et al. Among authors: schlemmer d. Biochem Biophys Res Commun. 2004 Apr 16;316(4):1138-42. doi: 10.1016/j.bbrc.2004.03.002. Biochem Biophys Res Commun. 2004. PMID: 15044103

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