407 related articles for article (PubMed ID: 34849584)
1. 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]
2. 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]
3. Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4
Yin Z; Agip AA; Bridges HR; Hirst J
EMBO J; 2024 Jan; 43(2):225-249. PubMed ID: 38177503
[TBL] [Abstract][Full Text] [Related]
4. 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]
5. The NDUFS4 Knockout Mouse: A Dual Threat Model of Childhood Mitochondrial Disease and Normative Aging.
Grillo AS; Bitto A; Kaeberlein M
Methods Mol Biol; 2021; 2277():143-155. PubMed ID: 34080150
[TBL] [Abstract][Full Text] [Related]
6. 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]
7. 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]
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. Photoreceptors in a mouse model of Leigh syndrome are capable of normal light-evoked signaling.
Gospe SM; Travis AM; Kolesnikov AV; Klingeborn M; Wang L; Kefalov VJ; Arshavsky VY
J Biol Chem; 2019 Aug; 294(33):12432-12443. PubMed ID: 31248988
[TBL] [Abstract][Full Text] [Related]
10. Iron status influences mitochondrial disease progression in Complex I-deficient mice.
Kelly CJ; Couch RK; Ha VT; Bodart CM; Wu J; Huff S; Herrel NT; Kim HD; Zimmermann AO; Shattuck J; Pan YC; Kaeberlein M; Grillo AS
Elife; 2023 Feb; 12():. PubMed ID: 36799301
[TBL] [Abstract][Full Text] [Related]
11. Metabolomics of Ndufs4
Terburgh K; Lindeque Z; Mason S; van der Westhuizen F; Louw R
Biochim Biophys Acta Mol Basis Dis; 2019 Jan; 1865(1):98-106. PubMed ID: 30391276
[TBL] [Abstract][Full Text] [Related]
12. Mutations in the ND5 subunit of complex I of the mitochondrial DNA are a frequent cause of oxidative phosphorylation disease.
Blok MJ; Spruijt L; de Coo IF; Schoonderwoerd K; Hendrickx A; Smeets HJ
J Med Genet; 2007 Apr; 44(4):e74. PubMed ID: 17400793
[TBL] [Abstract][Full Text] [Related]
13. A constant and similar assembly defect of mitochondrial respiratory chain complex I allows rapid identification of NDUFS4 mutations in patients with Leigh syndrome.
Assouline Z; Jambou M; Rio M; Bole-Feysot C; de Lonlay P; Barnerias C; Desguerre I; Bonnemains C; Guillermet C; Steffann J; Munnich A; Bonnefont JP; Rötig A; Lebre AS
Biochim Biophys Acta; 2012 Jun; 1822(6):1062-9. PubMed ID: 22326555
[TBL] [Abstract][Full Text] [Related]
14. NDUFS4 mutations cause Leigh syndrome with predominant brainstem involvement.
Leshinsky-Silver E; Lebre AS; Minai L; Saada A; Steffann J; Cohen S; Rötig A; Munnich A; Lev D; Lerman-Sagie T
Mol Genet Metab; 2009 Jul; 97(3):185-9. PubMed ID: 19364667
[TBL] [Abstract][Full Text] [Related]
15. Deletion of the Complex I Subunit NDUFS4 Adversely Modulates Cellular Differentiation.
Johnson J; Lee W; Frazier AE; Vaghjiani V; Laskowski A; Rodriguez AL; Cagnone GL; McKenzie M; White SJ; Nisbet DR; Thorburn DR; St John JC
Stem Cells Dev; 2016 Feb; 25(3):239-50. PubMed ID: 26608563
[TBL] [Abstract][Full Text] [Related]
16. 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]
17. 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]
18. 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]
19. Ndufs4 KO mice: A model to study comorbid mood disorders associated with mitochondrial dysfunction.
van Rensburg DJ; Lindeque Z; Harvey BH; Steyn SF
Pharmacol Biochem Behav; 2024 Jan; 234():173689. PubMed ID: 38070656
[TBL] [Abstract][Full Text] [Related]
20. 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]
[Next] [New Search]