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Items: 1 to 20 of 179

1.

Elevated glutaric acid levels in Dhtkd1-/Gcdh- double knockout mice challenge our current understanding of lysine metabolism.

Biagosch C, Ediga RD, Hensler SV, Faerberboeck M, Kuehn R, Wurst W, Meitinger T, Kölker S, Sauer S, Prokisch H.

Biochim Biophys Acta Mol Basis Dis. 2017 Sep;1863(9):2220-2228. doi: 10.1016/j.bbadis.2017.05.018. Epub 2017 May 22.

2.

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.

3.

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
4.

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.

PMID:
20923787
5.

Impairment of GABAergic system contributes to epileptogenesis in glutaric acidemia type I.

Vendramin Pasquetti M, Meier L, Loureiro S, Ganzella M, Junges B, Barbieri Caus L, Umpierrez Amaral A, Koeller DM, Goodman S, Woontner M, Gomes de Souza DO, Wajner M, Calcagnotto ME.

Epilepsia. 2017 Oct;58(10):1771-1781. doi: 10.1111/epi.13862. Epub 2017 Aug 1.

6.

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
7.

Clinical and molecular investigation in Chinese patients with glutaric aciduria type I.

Zhang Y, Li H, Ma R, Mei L, Wei X, Liang D, Wu L.

Clin Chim Acta. 2016 Jan 30;453:75-9. doi: 10.1016/j.cca.2015.12.003. Epub 2015 Dec 4.

PMID:
26656312
8.

The M405V allele of the glutaryl-CoA dehydrogenase gene is an important marker for glutaric aciduria type I (GA-I) low excretors.

Schillaci LA, Greene CL, Strovel E, Rispoli-Joines J, Spector E, Woontner M, Scharer G, Enns GM, Gallagher R, Zinn AB, McCandless SE, Hoppel CL, Goodman SI, Bedoyan JK.

Mol Genet Metab. 2016 Sep;119(1-2):50-6. doi: 10.1016/j.ymgme.2016.06.012. Epub 2016 Jul 1.

PMID:
27397597
9.

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.

10.

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
11.

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.

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.

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.

14.

Impairment of astrocytic glutaminolysis in glutaric aciduria type I.

Komatsuzaki S, Ediga RD, Okun JG, Kölker S, Sauer SW.

J Inherit Metab Dis. 2018 Jan;41(1):91-99. doi: 10.1007/s10545-017-0096-5. Epub 2017 Nov 2.

PMID:
29098534
15.

Understanding cerebral L-lysine metabolism: the role of L-pipecolate metabolism in Gcdh-deficient mice as a model for glutaric aciduria type I.

Posset R, Opp S, Struys EA, Völkl A, Mohr H, Hoffmann GF, Kölker S, Sauer SW, Okun JG.

J Inherit Metab Dis. 2015 Mar;38(2):265-72. doi: 10.1007/s10545-014-9762-z. Epub 2014 Sep 12.

PMID:
25214427
16.

(1)H-MRS in glutaric aciduria type 1: impact of biochemical phenotype and age on the cerebral accumulation of neurotoxic metabolites.

Harting I, Boy N, Heringer J, Seitz A, Bendszus M, Pouwels PJ, Kölker S.

J Inherit Metab Dis. 2015 Sep;38(5):829-38. doi: 10.1007/s10545-015-9826-8. Epub 2015 Apr 10.

PMID:
25860816
17.

Oxidative Stress, Disrupted Energy Metabolism, and Altered Signaling Pathways in Glutaryl-CoA Dehydrogenase Knockout Mice: Potential Implications of Quinolinic Acid Toxicity in the Neuropathology of Glutaric Acidemia Type I.

Seminotti B, Amaral AU, Ribeiro RT, Rodrigues MDN, Colín-González AL, Leipnitz G, Santamaría A, Wajner M.

Mol Neurobiol. 2016 Nov;53(9):6459-6475. doi: 10.1007/s12035-015-9548-9. Epub 2015 Nov 25.

PMID:
26607633
18.

[Progress of glutaric aciduria type I].

Gao JZ, Luo XP.

Zhonghua Er Ke Za Zhi. 2012 Dec;50(12):912-4. Review. Chinese. No abstract available.

PMID:
23324148
19.

[Complex heterogeneity phenotypes and genotypes of glutaric aciduria type 1].

Wang Q, Yang YL.

Zhongguo Dang Dai Er Ke Za Zhi. 2016 May;18(5):460-5. Review. Chinese.

20.

Induction of Neuroinflammatory Response and Histopathological Alterations Caused by Quinolinic Acid Administration in the Striatum of Glutaryl-CoA Dehydrogenase Deficient Mice.

Amaral AU, Seminotti B, da Silva JC, de Oliveira FH, Ribeiro RT, Vargas CR, Leipnitz G, Santamaría A, Souza DO, Wajner M.

Neurotox Res. 2018 Apr;33(3):593-606. doi: 10.1007/s12640-017-9848-0. Epub 2017 Dec 12.

PMID:
29235064

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