206 related articles for article (PubMed ID: 36799301)
1. 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]
2. 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]
3. 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]
4. mTOR inhibition alleviates mitochondrial disease in a mouse model of Leigh syndrome.
Johnson SC; Yanos ME; Kayser EB; Quintana A; Sangesland M; Castanza A; Uhde L; Hui J; Wall VZ; Gagnidze A; Oh K; Wasko BM; Ramos FJ; Palmiter RD; Rabinovitch PS; Morgan PG; Sedensky MM; Kaeberlein M
Science; 2013 Dec; 342(6165):1524-8. PubMed ID: 24231806
[TBL] [Abstract][Full Text] [Related]
5. 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]
6. 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]
7. 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]
8. 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]
9. Mitochondrial defects caused by PARL deficiency lead to arrested spermatogenesis and ferroptosis.
Radaelli E; Assenmacher CA; Verrelle J; Banerjee E; Manero F; Khiati S; Girona A; Lopez-Lluch G; Navas P; Spinazzi M
Elife; 2023 Jul; 12():. PubMed ID: 37505079
[TBL] [Abstract][Full Text] [Related]
10. 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]
11. 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]
12. Aberrant BCAA and glutamate metabolism linked to regional neurodegeneration in a mouse model of Leigh syndrome.
Terburgh K; Coetzer J; Lindeque JZ; van der Westhuizen FH; Louw R
Biochim Biophys Acta Mol Basis Dis; 2021 May; 1867(5):166082. PubMed ID: 33486097
[TBL] [Abstract][Full Text] [Related]
13. The Blood-Brain Barrier Is Unaffected in the
Reynaud-Dulaurier R; Clément R; Yjjou S; Cresson C; Saoudi Y; Faideau M; Decressac M
Int J Mol Sci; 2024 Apr; 25(9):. PubMed ID: 38732047
[TBL] [Abstract][Full Text] [Related]
14. Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain.
Kayser EB; Sedensky MM; Morgan PG
PLoS One; 2016; 11(1):e0148219. PubMed ID: 26824698
[TBL] [Abstract][Full Text] [Related]
15. 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]
16. 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]
17. 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]
18. 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]
19. 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]
20. 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]
[Next] [New Search]