151 related articles for article (PubMed ID: 26363424)
1. Gait analysis in a mouse model resembling Leigh disease.
de Haas R; Russel FG; Smeitink JA
Behav Brain Res; 2016 Jan; 296():191-198. PubMed ID: 26363424
[TBL] [Abstract][Full Text] [Related]
2. Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention.
van de Wal MAE; Adjobo-Hermans MJW; Keijer J; Schirris TJJ; Homberg JR; Wieckowski MR; Grefte S; van Schothorst EM; van Karnebeek C; Quintana A; Koopman WJH
Brain; 2022 Mar; 145(1):45-63. PubMed ID: 34849584
[TBL] [Abstract][Full Text] [Related]
3. Fatal breathing dysfunction in a mouse model of Leigh syndrome.
Quintana A; Zanella S; Koch H; Kruse SE; Lee D; Ramirez JM; Palmiter RD
J Clin Invest; 2012 Jul; 122(7):2359-68. PubMed ID: 22653057
[TBL] [Abstract][Full Text] [Related]
4. Interferon-gamma contributes to disease progression in the Ndufs4(-/-) model of Leigh syndrome.
Hanaford AR; Khanna A; James K; Truong V; Liao R; Chen Y; Mulholland M; Kayser EB; Watanabe K; Hsieh ES; Sedensky M; Morgan PG; Kalia V; Sarkar S; Johnson SC
Neuropathol Appl Neurobiol; 2024 Jun; 50(3):e12977. PubMed ID: 38680020
[TBL] [Abstract][Full Text] [Related]
5. Impaired hypoxic pulmonary vasoconstriction in a mouse model of Leigh syndrome.
Schleifer G; Marutani E; Ferrari M; Sharma R; Skinner O; Goldberger O; Grange RMH; Peneyra K; Malhotra R; Wepler M; Ichinose F; Bloch DB; Mootha VK; Zapol WM
Am J Physiol Lung Cell Mol Physiol; 2019 Feb; 316(2):L391-L399. PubMed ID: 30520688
[TBL] [Abstract][Full Text] [Related]
6. Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome.
Aguilar K; Comes G; Canal C; Quintana A; Sanz E; Hidalgo J
Glia; 2022 Nov; 70(11):2032-2044. PubMed ID: 35770802
[TBL] [Abstract][Full Text] [Related]
7. Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome.
Ferrari M; Jain IH; Goldberger O; Rezoagli E; Thoonen R; Cheng KH; Sosnovik DE; Scherrer-Crosbie M; Mootha VK; Zapol WM
Proc Natl Acad Sci U S A; 2017 May; 114(21):E4241-E4250. PubMed ID: 28483998
[TBL] [Abstract][Full Text] [Related]
8. Double administration of self-complementary AAV9NDUFS4 prevents Leigh disease in Ndufs4-/- mice.
Corrà S; Cerutti R; Balmaceda V; Viscomi C; Zeviani M
Brain; 2022 Oct; 145(10):3405-3414. PubMed ID: 36270002
[TBL] [Abstract][Full Text] [Related]
9. Proteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element (SINE) insertion into NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) gene.
Leong DW; Komen JC; Hewitt CA; Arnaud E; McKenzie M; Phipson B; Bahlo M; Laskowski A; Kinkel SA; Davey GM; Heath WR; Voss AK; Zahedi RP; Pitt JJ; Chrast R; Sickmann A; Ryan MT; Smyth GK; Thorburn DR; Scott HS
J Biol Chem; 2012 Jun; 287(24):20652-63. PubMed ID: 22535952
[TBL] [Abstract][Full Text] [Related]
10. Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease.
Johnson SC; Kayser EB; Bornstein R; Stokes J; Bitto A; Park KY; Pan A; Sun G; Raftery D; Kaeberlein M; Sedensky MM; Morgan PG
Mol Genet Metab; 2020 Jun; 130(2):118-132. PubMed ID: 32331968
[TBL] [Abstract][Full Text] [Related]
11. Succination is Increased on Select Proteins in the Brainstem of the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) Knockout Mouse, a Model of Leigh Syndrome.
Piroli GG; Manuel AM; Clapper AC; Walla MD; Baatz JE; Palmiter RD; Quintana A; Frizzell N
Mol Cell Proteomics; 2016 Feb; 15(2):445-61. PubMed ID: 26450614
[TBL] [Abstract][Full Text] [Related]
12. Acarbose suppresses symptoms of mitochondrial disease in a mouse model of Leigh syndrome.
Bitto A; Grillo AS; Ito TK; Stanaway IB; Nguyen BMG; Ying K; Tung H; Smith K; Tran N; Velikanje G; Urfer SR; Snyder JM; Barton J; Sharma A; Kayser EB; Wang L; Smith DL; Thompson JW; DuBois L; DePaolo W; Kaeberlein M
Nat Metab; 2023 Jun; 5(6):955-967. PubMed ID: 37365290
[TBL] [Abstract][Full Text] [Related]
13. Hypoxia ameliorates brain hyperoxia and NAD
Grange RMH; Sharma R; Shah H; Reinstadler B; Goldberger O; Cooper MK; Nakagawa A; Miyazaki Y; Hindle AG; Batten AJ; Wojtkiewicz GR; Schleifer G; Bagchi A; Marutani E; Malhotra R; Bloch DB; Ichinose F; Mootha VK; Zapol WM
Mol Genet Metab; 2021 May; 133(1):83-93. PubMed ID: 33752971
[TBL] [Abstract][Full Text] [Related]
14. Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease.
de Haas R; Das D; Garanto A; Renkema HG; Greupink R; van den Broek P; Pertijs J; Collin RWJ; Willems P; Beyrath J; Heerschap A; Russel FG; Smeitink JA
Sci Rep; 2017 Sep; 7(1):11733. PubMed ID: 28916769
[TBL] [Abstract][Full Text] [Related]
15. Targeting NAD
Lee CF; Caudal A; Abell L; Nagana Gowda GA; Tian R
Sci Rep; 2019 Feb; 9(1):3073. PubMed ID: 30816177
[TBL] [Abstract][Full Text] [Related]
16. NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4
Adjobo-Hermans MJW; de Haas R; Willems PHGM; Wojtala A; van Emst-de Vries SE; Wagenaars JA; van den Brand M; Rodenburg RJ; Smeitink JAM; Nijtmans LG; Sazanov LA; Wieckowski MR; Koopman WJH
Biochim Biophys Acta Bioenerg; 2020 Aug; 1861(8):148213. PubMed ID: 32335026
[TBL] [Abstract][Full Text] [Related]
17. Defined neuronal populations drive fatal phenotype in a mouse model of Leigh syndrome.
Bolea I; Gella A; Sanz E; Prada-Dacasa P; Menardy F; Bard AM; Machuca-Márquez P; Eraso-Pichot A; Mòdol-Caballero G; Navarro X; Kalume F; Quintana A
Elife; 2019 Aug; 8():. PubMed ID: 31403401
[TBL] [Abstract][Full Text] [Related]
18. Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice.
Frambach SJCM; van de Wal MAE; van den Broek PHH; Smeitink JAM; Russel FGM; de Haas R; Schirris TJJ
Biochim Biophys Acta Mol Basis Dis; 2020 Jun; 1866(6):165727. PubMed ID: 32070771
[TBL] [Abstract][Full Text] [Related]
19. Elevated susceptibility to exogenous seizure triggers and impaired interneuron excitability in a mouse model of Leigh syndrome epilepsy.
Manning A; Han V; Stephens A; Wang R; Bush N; Bard M; Ramirez JM; Kalume F
Neurobiol Dis; 2023 Oct; 187():106288. PubMed ID: 37704057
[TBL] [Abstract][Full Text] [Related]
20. Mitochondrial complex I deficiency leads to the retardation of early embryonic development in
Wang M; Huang YP; Wu H; Song K; Wan C; Chi AN; Xiao YM; Zhao XY
PeerJ; 2017; 5():e3339. PubMed ID: 28533980
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
