Sauer SW - Search Results

1

Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins.

Schwab MA, Sauer SW, Okun JG, Nijtmans LG, Rodenburg RJ, van den Heuvel LP, Dröse S, Brandt U, Hoffmann GF, Ter Laak H, Kölker S, Smeitink JA. Schwab MA, et al. Among authors: sauer sw. Biochem J. 2006 Aug 15;398(1):107-12. doi: 10.1042/BJ20060221. Biochem J. 2006. PMID: 16686602 Free PMC article.

2

Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity.

Okun JG, Hörster F, Farkas LM, Feyh P, Hinz A, Sauer S, Hoffmann GF, Unsicker K, Mayatepek E, Kölker S. Okun JG, et al. J Biol Chem. 2002 Apr 26;277(17):14674-80. doi: 10.1074/jbc.M200997200. Epub 2002 Feb 14. J Biol Chem. 2002. PMID: 11847233 Free article.

3

Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain.

Kölker S, Schwab M, Hörster F, Sauer S, Hinz A, Wolf NI, Mayatepek E, Hoffmann GF, Smeitink JA, Okun JG. Kölker S, et al. J Biol Chem. 2003 Nov 28;278(48):47388-93. doi: 10.1074/jbc.M308861200. Epub 2003 Sep 12. J Biol Chem. 2003. PMID: 12972416 Free article.

4

Excitotoxicity and bioenergetics in glutaryl-CoA dehydrogenase deficiency.

Kölker S, Koeller DM, Sauer S, Hörster F, Schwab MA, Hoffmann GF, Ullrich K, Okun JG. Kölker S, et al. J Inherit Metab Dis. 2004;27(6):805-12. doi: 10.1023/B:BOLI.0000045762.37248.28. J Inherit Metab Dis. 2004. PMID: 15505385 Review.

5

Animal models for glutaryl-CoA dehydrogenase deficiency.

Koeller DM, Sauer S, Wajner M, de Mello CF, Goodman SI, Woontner M, Mühlhausen C, Okun JG, Kölker S. Koeller DM, et al. J Inherit Metab Dis. 2004;27(6):813-8. doi: 10.1023/B:BOLI.0000045763.52907.5e. J Inherit Metab Dis. 2004. PMID: 15505386

6

Optimized spectrophotometric assay for the completely activated pyruvate dehydrogenase complex in fibroblasts.

Schwab MA, Kölker S, van den Heuvel LP, Sauer S, Wolf NI, Rating D, Hoffmann GF, Smeitink JA, Okun JG. Schwab MA, et al. Clin Chem. 2005 Jan;51(1):151-60. doi: 10.1373/clinchem.2004.033852. Epub 2004 Nov 18. Clin Chem. 2005. PMID: 15550478

7

S-Acetylglutathione normalizes intracellular glutathione content in cultured fibroblasts from patients with glutathione synthetase deficiency.

Okun JG, Sauer S, Bähr S, Lenhartz H, Mayatepek E. Okun JG, et al. J Inherit Metab Dis. 2004;27(6):783-6. doi: 10.1023/b:boli.0000045838.65498.ff. J Inherit Metab Dis. 2004. PMID: 15617191

8

Neuropathological, biochemical and molecular findings in a glutaric acidemia type 1 cohort.

Funk CB, Prasad AN, Frosk P, Sauer S, Kölker S, Greenberg CR, Del Bigio MR. Funk CB, et al. Brain. 2005 Apr;128(Pt 4):711-22. doi: 10.1093/brain/awh401. Epub 2005 Feb 2. Brain. 2005. PMID: 15689364

9

Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A.

Sauer SW, Okun JG, Schwab MA, Crnic LR, Hoffmann GF, Goodman SI, Koeller DM, Kölker S. Sauer SW, et al. J Biol Chem. 2005 Jun 10;280(23):21830-6. doi: 10.1074/jbc.M502845200. Epub 2005 Apr 19. J Biol Chem. 2005. PMID: 15840571 Free article.

10

Late-onset neurologic disease in glutaryl-CoA dehydrogenase deficiency.

Külkens S, Harting I, Sauer S, Zschocke J, Hoffmann GF, Gruber S, Bodamer OA, Kölker S. Külkens S, et al. Neurology. 2005 Jun 28;64(12):2142-4. doi: 10.1212/01.WNL.0000167428.12417.B2. Neurology. 2005. PMID: 15985591

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