Okun J - Search Results

1

Human heterologous liver cells transiently improve hyperammonemia and ureagenesis in individuals with severe urea cycle disorders.

Meyburg J, Opladen T, Spiekerkötter U, Schlune A, Schenk JP, Schmidt J, Weitz J, Okun J, Bürger F, Omran TB, Abdoh G, Al Rifai H, Monavari A, Konstantopoulou V, Kölker S, Yudkoff M, Hoffmann GF. Meyburg J, et al. Among authors: okun j. J Inherit Metab Dis. 2018 Jan;41(1):81-90. doi: 10.1007/s10545-017-0097-4. Epub 2017 Oct 12. J Inherit Metab Dis. 2018. PMID: 29027067 Clinical Trial.

2

3-Ureidopropionate contributes to the neuropathology of 3-ureidopropionase deficiency and severe propionic aciduria: a hypothesis.

Kölker S, Okun JG, Hörster F, Assmann B, Ahlemeyer B, Kohlmüller D, Exner-Camps S, Mayatepek E, Krieglstein J, Hoffmann GF. Kölker S, et al. Among authors: okun jg. J Neurosci Res. 2001 Nov 15;66(4):666-73. doi: 10.1002/jnr.10012. J Neurosci Res. 2001. PMID: 11746386

3

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.

4

Chronic treatment with glutaric acid induces partial tolerance to excitotoxicity in neuronal cultures from chick embryo telencephalons.

Kölker S, Okun JG, Ahlemeyer B, Wyse AT, Hörster F, Wajner M, Kohlmüller D, Mayatepek E, Krieglstein J, Hoffmann GF. Kölker S, et al. Among authors: okun jg. J Neurosci Res. 2002 May 15;68(4):424-31. doi: 10.1002/jnr.10189. J Neurosci Res. 2002. PMID: 11992468

5

Ca(2+) and Na(+) dependence of 3-hydroxyglutarate-induced excitotoxicity in primary neuronal cultures from chick embryo telencephalons.

Kölker S, Köhr G, Ahlemeyer B, Okun JG, Pawlak V, Hörster F, Mayatepek E, Krieglstein J, Hoffmann GF. Kölker S, et al. Among authors: okun jg. Pediatr Res. 2002 Aug;52(2):199-206. doi: 10.1203/00006450-200208000-00011. Pediatr Res. 2002. PMID: 12149496

6

NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration in d-2-hydroxyglutaric aciduria.

Kölker S, Pawlak V, Ahlemeyer B, Okun JG, Hörster F, Mayatepek E, Krieglstein J, Hoffmann GF, Köhr G. Kölker S, et al. Among authors: okun jg. Eur J Neurosci. 2002 Jul;16(1):21-8. doi: 10.1046/j.1460-9568.2002.02055.x. Eur J Neurosci. 2002. PMID: 12153528

7

A method for quantitative acylcarnitine profiling in human skin fibroblasts using unlabelled palmitic acid: diagnosis of fatty acid oxidation disorders and differentiation between biochemical phenotypes of MCAD deficiency.

Okun JG, Kölker S, Schulze A, Kohlmüller D, Olgemöller K, Lindner M, Hoffmann GF, Wanders RJ, Mayatepek E. Okun JG, et al. Biochim Biophys Acta. 2002 Oct 10;1584(2-3):91-8. doi: 10.1016/s1388-1981(02)00296-2. Biochim Biophys Acta. 2002. PMID: 12385891

8

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.

9

Glutaryl-CoA dehydrogenase deficiency: region-specific analysis of organic acids and acylcarnitines in post mortem brain predicts vulnerability of the putamen.

Kölker S, Hoffmann GF, Schor DS, Feyh P, Wagner L, Jeffrey I, Pourfarzam M, Okun JG, Zschocke J, Baric I, Bain MD, Jakobs C, Chalmers RA. Kölker S, et al. Among authors: okun jg. Neuropediatrics. 2003 Jun;34(5):253-60. doi: 10.1055/s-2003-43261. Neuropediatrics. 2003. PMID: 14598231

10

Pathomechanisms of neurodegeneration in glutaryl-CoA dehydrogenase deficiency.

Kölker S, Koeller DM, Okun JG, Hoffmann GF. Kölker S, et al. Ann Neurol. 2004 Jan;55(1):7-12. doi: 10.1002/ana.10784. Ann Neurol. 2004. PMID: 14705106 Review.

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