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Items: 1 to 20 of 272

1.

Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency.

Sauer SW, Okun JG, Fricker G, Mahringer A, Müller I, Crnic LR, Mühlhausen C, Hoffmann GF, Hörster F, Goodman SI, Harding CO, Koeller DM, Kölker S.

J Neurochem. 2006 May;97(3):899-910. Epub 2006 Mar 29.

2.

Glutaric aciduria type I and methylmalonic aciduria: simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood-brain barrier and the choroid plexus.

Sauer SW, Opp S, Mahringer A, Kamiński MM, Thiel C, Okun JG, Fricker G, Morath MA, Kölker S.

Biochim Biophys Acta. 2010 Jun;1802(6):552-60. doi: 10.1016/j.bbadis.2010.03.003. Epub 2010 Mar 17.

3.

Biochemistry and bioenergetics of glutaryl-CoA dehydrogenase deficiency.

Sauer SW.

J Inherit Metab Dis. 2007 Oct;30(5):673-80. Epub 2007 Sep 21.

PMID:
17879145
4.

Transport and distribution of 3-hydroxyglutaric acid before and during induced encephalopathic crises in a mouse model of glutaric aciduria type 1.

Keyser B, Glatzel M, Stellmer F, Kortmann B, Lukacs Z, Kölker S, Sauer SW, Muschol N, Herdering W, Thiem J, Goodman SI, Koeller DM, Ullrich K, Braulke T, Mühlhausen C.

Biochim Biophys Acta. 2008 Jun;1782(6):385-90. doi: 10.1016/j.bbadis.2008.02.008. Epub 2008 Feb 29.

5.

Therapeutic modulation of cerebral L-lysine metabolism in a mouse model for glutaric aciduria type I.

Sauer SW, Opp S, Hoffmann GF, Koeller DM, Okun JG, Kölker S.

Brain. 2011 Jan;134(Pt 1):157-70. doi: 10.1093/brain/awq269. Epub 2010 Oct 4.

6.

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.

Neuropediatrics. 2003 Jun;34(5):253-60.

PMID:
14598231
7.

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.

J Inherit Metab Dis. 2004;27(6):813-8.

PMID:
15505386
8.

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.

J Biol Chem. 2005 Jun 10;280(23):21830-6. Epub 2005 Apr 19.

9.

Multifactorial modulation of susceptibility to l-lysine in an animal model of glutaric aciduria type I.

Sauer SW, Opp S, Komatsuzaki S, Blank AE, Mittelbronn M, Burgard P, Koeller DM, Okun JG, Kölker S.

Biochim Biophys Acta. 2015 May;1852(5):768-77. doi: 10.1016/j.bbadis.2014.12.022. Epub 2015 Jan 2.

10.

Biochemical, pathologic and behavioral analysis of a mouse model of glutaric acidemia type I.

Koeller DM, Woontner M, Crnic LS, Kleinschmidt-DeMasters B, Stephens J, Hunt EL, Goodman SI.

Hum Mol Genet. 2002 Feb 15;11(4):347-57.

11.

A diet-induced mouse model for glutaric aciduria type I.

Zinnanti WJ, Lazovic J, Wolpert EB, Antonetti DA, Smith MB, Connor JR, Woontner M, Goodman SI, Cheng KC.

Brain. 2006 Apr;129(Pt 4):899-910. Epub 2006 Jan 30.

12.

3-Hydroxyglutaric acid is transported via the sodium-dependent dicarboxylate transporter NaDC3.

Stellmer F, Keyser B, Burckhardt BC, Koepsell H, Streichert T, Glatzel M, Jabs S, Thiem J, Herdering W, Koeller DM, Goodman SI, Lukacs Z, Ullrich K, Burckhardt G, Braulke T, Mühlhausen C.

J Mol Med (Berl). 2007 Jul;85(7):763-70. Epub 2007 Mar 14.

PMID:
17356845
13.

Acute renal proximal tubule alterations during induced metabolic crises in a mouse model of glutaric aciduria type 1.

Thies B, Meyer-Schwesinger C, Lamp J, Schweizer M, Koeller DM, Ullrich K, Braulke T, Mühlhausen C.

Biochim Biophys Acta. 2013 Oct;1832(10):1463-72. doi: 10.1016/j.bbadis.2013.04.019. Epub 2013 Apr 24.

14.

Infant mice with glutaric acidaemia type I have increased vulnerability to 3-nitropropionic acid toxicity.

Bjugstad KB, Crnic LS, Goodman SI, Freed CR.

J Inherit Metab Dis. 2006 Oct;29(5):612-9. Epub 2006 Aug 30.

PMID:
16944278
15.

Disturbance of the glutamatergic system by glutaric acid in striatum and cerebral cortex of glutaryl-CoA dehydrogenase-deficient knockout mice: possible implications for the neuropathology of glutaric acidemia type I.

Busanello EN, Fernandes CG, Martell RV, Lobato VG, Goodman S, Woontner M, de Souza DO, Wajner M.

J Neurol Sci. 2014 Nov 15;346(1-2):260-7. doi: 10.1016/j.jns.2014.09.003. Epub 2014 Sep 16.

PMID:
25241940
16.

On the neurotoxicity of glutaric, 3-hydroxyglutaric, and trans-glutaconic acids in glutaric acidemia type 1.

Lund TM, Christensen E, Kristensen AS, Schousboe A, Lund AM.

J Neurosci Res. 2004 Jul 1;77(1):143-7.

PMID:
15197747
17.

Marked reduction of Na(+), K(+)-ATPase and creatine kinase activities induced by acute lysine administration in glutaryl-CoA dehydrogenase deficient mice.

Amaral AU, Cecatto C, Seminotti B, Zanatta Â, Fernandes CG, Busanello EN, Braga LM, Ribeiro CA, de Souza DO, Woontner M, Koeller DM, Goodman S, Wajner M.

Mol Genet Metab. 2012 Sep;107(1-2):81-6. doi: 10.1016/j.ymgme.2012.04.015. Epub 2012 Apr 24.

PMID:
22578804
18.

Glutaric aciduria type 1 metabolites impair the succinate transport from astrocytic to neuronal cells.

Lamp J, Keyser B, Koeller DM, Ullrich K, Braulke T, Mühlhausen C.

J Biol Chem. 2011 May 20;286(20):17777-84. doi: 10.1074/jbc.M111.232744. Epub 2011 Mar 29.

19.

Induction of oxidative stress in brain of glutaryl-CoA dehydrogenase deficient mice by acute lysine administration.

Seminotti B, da Rosa MS, Fernandes CG, Amaral AU, Braga LM, Leipnitz G, de Souza DO, Woontner M, Koeller DM, Goodman S, Wajner M.

Mol Genet Metab. 2012 May;106(1):31-8. doi: 10.1016/j.ymgme.2012.03.002. Epub 2012 Mar 13.

PMID:
22445450
20.

Subependymal mass lesions and peripheral polyneuropathy in adult-onset glutaric aciduria type I.

Herskovitz M, Goldsher D, Sela BA, Mandel H.

Neurology. 2013 Aug 27;81(9):849-50. doi: 10.1212/WNL.0b013e3182a2cbf2. Epub 2013 Jul 24.

PMID:
23884036
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