244 related articles for article (PubMed ID: 21454630)
1. 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; 286(20):17777-84. PubMed ID: 21454630
[TBL] [Abstract][Full Text] [Related]
2. 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. PubMed ID: 18404412
[TBL] [Abstract][Full Text] [Related]
3. 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. PubMed ID: 23623985
[TBL] [Abstract][Full Text] [Related]
4. 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; 24(16):4504-15. PubMed ID: 25968119
[TBL] [Abstract][Full Text] [Related]
5. 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. PubMed ID: 28545977
[TBL] [Abstract][Full Text] [Related]
6. 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. PubMed ID: 25558815
[TBL] [Abstract][Full Text] [Related]
7. 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. PubMed ID: 29098534
[TBL] [Abstract][Full Text] [Related]
8. 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; 346(1-2):260-7. PubMed ID: 25241940
[TBL] [Abstract][Full Text] [Related]
9. The first knock-in rat model for glutaric aciduria type I allows further insights into pathophysiology in brain and periphery.
Gonzalez Melo M; Remacle N; Cudré-Cung HP; Roux C; Poms M; Cudalbu C; Barroso M; Gersting SW; Feichtinger RG; Mayr JA; Costanzo M; Caterino M; Ruoppolo M; Rüfenacht V; Häberle J; Braissant O; Ballhausen D
Mol Genet Metab; 2021 Jun; 133(2):157-181. PubMed ID: 33965309
[TBL] [Abstract][Full Text] [Related]
10. 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. PubMed ID: 18348873
[TBL] [Abstract][Full Text] [Related]
11. Deletion of 2-aminoadipic semialdehyde synthase limits metabolite accumulation in cell and mouse models for glutaric aciduria type 1.
Leandro J; Dodatko T; DeVita RJ; Chen H; Stauffer B; Yu C; Houten SM
J Inherit Metab Dis; 2020 Nov; 43(6):1154-1164. PubMed ID: 32567100
[TBL] [Abstract][Full Text] [Related]
12. 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; 9(10):e110181. PubMed ID: 25333616
[TBL] [Abstract][Full Text] [Related]
13. Induction of S100B secretion in C6 astroglial cells by the major metabolites accumulating in glutaric acidemia type I.
Quincozes-Santos A; Rosa RB; Leipnitz G; de Souza DF; Seminotti B; Wajner M; Gonçalves CA
Metab Brain Dis; 2010 Jun; 25(2):191-8. PubMed ID: 20437086
[TBL] [Abstract][Full Text] [Related]
14. 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; 453():75-9. PubMed ID: 26656312
[TBL] [Abstract][Full Text] [Related]
15. Adult-onset glutaric aciduria type I: rare presentation of a treatable disorder.
Gelener P; Severino M; Diker S; Teralı K; Tuncel G; Tuzlalı H; Manara E; Paolacci S; Bertelli M; Ergoren MC
Neurogenetics; 2020 Jul; 21(3):179-186. PubMed ID: 32306145
[TBL] [Abstract][Full Text] [Related]
16. 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. PubMed ID: 28762469
[TBL] [Abstract][Full Text] [Related]
17. 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. PubMed ID: 23326493
[TBL] [Abstract][Full Text] [Related]
18. An explanation for metabolite excretion in high- and low-excretor patients with glutaric acidemia type 1.
Goodman SI; Woontner M
Mol Genet Metab; 2019 Aug; 127(4):325-326. PubMed ID: 31324527
[No Abstract] [Full Text] [Related]
19. Effects of targeted suppression of glutaryl-CoA dehydrogenase by lentivirus-mediated shRNA and excessive intake of lysine on apoptosis in rat striatal neurons.
Gao J; Zhang C; Fu X; Yi Q; Tian F; Ning Q; Luo X
PLoS One; 2013; 8(5):e63084. PubMed ID: 23658800
[TBL] [Abstract][Full Text] [Related]
20. 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. PubMed ID: 27397597
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]