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Items: 1 to 20 of 115

1.

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.

2.

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.

3.

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.

4.

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.

5.

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
6.

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.

7.

Interaction of glutaric aciduria type 1-related glutaryl-CoA dehydrogenase with mitochondrial matrix proteins.

Schmiesing J, Schlüter H, Ullrich K, Braulke T, Mühlhausen C.

PLoS One. 2014 Feb 3;9(2):e87715. doi: 10.1371/journal.pone.0087715. eCollection 2014.

8.

Experimental evidence that bioenergetics disruption is not mainly involved in the brain injury of glutaryl-CoA dehydrogenase deficient mice submitted to lysine overload.

Amaral AU, Cecatto C, Seminotti B, Ribeiro CA, Lagranha VL, Pereira CC, de Oliveira FH, de Souza DG, Goodman S, Woontner M, Wajner M.

Brain Res. 2015 Sep 16;1620:116-29. doi: 10.1016/j.brainres.2015.05.013. Epub 2015 May 18.

PMID:
25998543
9.

Membrane translocation of glutaric acid and its derivatives.

Mühlhausen C, Burckhardt BC, Hagos Y, Burckhardt G, Keyser B, Lukacs Z, Ullrich K, Braulke T.

J Inherit Metab Dis. 2008 Apr;31(2):188-93. doi: 10.1007/s10545-008-0825-x. Epub 2008 Apr 4. Review.

PMID:
18404412
10.

Striatal neuronal death mediated by astrocytes from the Gcdh-/- mouse model of glutaric acidemia type I.

Olivera-Bravo S, Ribeiro CA, Isasi E, Trías E, Leipnitz G, Díaz-Amarilla P, Woontner M, Beck C, Goodman SI, Souza D, Wajner M, Barbeito L.

Hum Mol Genet. 2015 Aug 15;24(16):4504-15. doi: 10.1093/hmg/ddv175. Epub 2015 May 12.

PMID:
25968119
11.

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
12.

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
13.

Mechanistic effects of amino acids and glucose in a novel glutaric aciduria type 1 cell model.

Fu X, Gao H, Tian F, Gao J, Lou L, Liang Y, Ning Q, Luo X.

PLoS One. 2014 Oct 15;9(10):e110181. doi: 10.1371/journal.pone.0110181. eCollection 2014.

14.

Glutaric Aciduria Type 1 and Acute Renal Failure: Case Report and Suggested Pathomechanisms.

du Moulin M, Thies B, Blohm M, Oh J, Kemper MJ, Santer R, Mühlhausen C.

JIMD Rep. 2017 Jul 12. doi: 10.1007/8904_2017_44. [Epub ahead of print]

PMID:
28699143
15.

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.

PMID:
16446282
16.

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
17.

A Korean patient with glutaric aciduria type 1 with a novel mutation in the glutaryl CoA dehydrogenase gene.

Kim HS, Yu HJ, Lee J, Park HD, Kim JH, Shin HJ, Jin DK, Lee M.

Ann Clin Lab Sci. 2014 Spring;44(2):213-6.

PMID:
24795062
18.

Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH).

Keyser B, Mühlhausen C, Dickmanns A, Christensen E, Muschol N, Ullrich K, Braulke T.

Hum Mol Genet. 2008 Dec 15;17(24):3854-63. doi: 10.1093/hmg/ddn284. Epub 2008 Sep 5.

PMID:
18775954
19.

Experimental evidence that overexpression of NR2B glutamate receptor subunit is associated with brain vacuolation in adult glutaryl-CoA dehydrogenase deficient mice: A potential role for glutamatergic-induced excitotoxicity in GA I neuropathology.

Rodrigues MD, Seminotti B, Amaral AU, Leipnitz G, Goodman SI, Woontner M, de Souza DO, Wajner M.

J Neurol Sci. 2015 Dec 15;359(1-2):133-40. doi: 10.1016/j.jns.2015.10.043. Epub 2015 Oct 28.

PMID:
26671102
20.

Ammonium accumulation and cell death in a rat 3D brain cell model of glutaric aciduria type I.

Jafari P, Braissant O, Zavadakova P, Henry H, Bonafé L, Ballhausen D.

PLoS One. 2013;8(1):e53735. doi: 10.1371/journal.pone.0053735. Epub 2013 Jan 10.

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