BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

221 related articles for article (PubMed ID: 33462515)

  • 21. 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]  

  • 22. 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]  

  • 23. Differential effects of mTOR inhibition and dietary ketosis in a mouse model of subacute necrotizing encephalomyelopathy.
    Bornstein R; James K; Stokes J; Park KY; Kayser EB; Snell J; Bard A; Chen Y; Kalume F; Johnson SC
    Neurobiol Dis; 2022 Feb; 163():105594. PubMed ID: 34933094
    [TBL] [Abstract][Full Text] [Related]  

  • 24. 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]  

  • 25. 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]  

  • 26. Cross-comparison of systemic and tissue-specific metabolomes in a mouse model of Leigh syndrome.
    Terburgh K; Lindeque JZ; van der Westhuizen FH; Louw R
    Metabolomics; 2021 Nov; 17(12):101. PubMed ID: 34792662
    [TBL] [Abstract][Full Text] [Related]  

  • 27. 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]  

  • 28. Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.
    Blanchet L; Smeitink JA; van Emst-de Vries SE; Vogels C; Pellegrini M; Jonckheere AI; Rodenburg RJ; Buydens LM; Beyrath J; Willems PH; Koopman WJ
    Sci Rep; 2015 Jan; 5():8035. PubMed ID: 25620325
    [TBL] [Abstract][Full Text] [Related]  

  • 29. Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome.
    Quintana A; Kruse SE; Kapur RP; Sanz E; Palmiter RD
    Proc Natl Acad Sci U S A; 2010 Jun; 107(24):10996-1001. PubMed ID: 20534480
    [TBL] [Abstract][Full Text] [Related]  

  • 30. Bromodomain Inhibitors Correct Bioenergetic Deficiency Caused by Mitochondrial Disease Complex I Mutations.
    Barrow JJ; Balsa E; Verdeguer F; Tavares CD; Soustek MS; Hollingsworth LR; Jedrychowski M; Vogel R; Paulo JA; Smeitink J; Gygi SP; Doench J; Root DE; Puigserver P
    Mol Cell; 2016 Oct; 64(1):163-175. PubMed ID: 27666594
    [TBL] [Abstract][Full Text] [Related]  

  • 31. Detection of increased intracerebral lactate in a mouse model of Leigh syndrome using proton MR spectroscopy.
    Takahashi Y; Kioka H; Shintani Y; Ohki A; Takashima S; Sakata Y; Higuchi T; Saito S
    Magn Reson Imaging; 2019 May; 58():38-43. PubMed ID: 30668983
    [TBL] [Abstract][Full Text] [Related]  

  • 32. Multisystem mitochondrial diseases due to mutations in mtDNA-encoded subunits of complex I.
    Danhelovska T; Kolarova H; Zeman J; Hansikova H; Vaneckova M; Lambert L; Kucerova-Vidrova V; Berankova K; Honzik T; Tesarova M
    BMC Pediatr; 2020 Jan; 20(1):41. PubMed ID: 31996177
    [TBL] [Abstract][Full Text] [Related]  

  • 33. The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome.
    Kanabus M; Fassone E; Hughes SD; Bilooei SF; Rutherford T; Donnell MO; Heales SJR; Rahman S
    J Inherit Metab Dis; 2016 May; 39(3):415-426. PubMed ID: 27080638
    [TBL] [Abstract][Full Text] [Related]  

  • 34. N-acetylcysteine and vitamin E rescue animal longevity and cellular oxidative stress in pre-clinical models of mitochondrial complex I disease.
    Polyak E; Ostrovsky J; Peng M; Dingley SD; Tsukikawa M; Kwon YJ; McCormack SE; Bennett M; Xiao R; Seiler C; Zhang Z; Falk MJ
    Mol Genet Metab; 2018 Apr; 123(4):449-462. PubMed ID: 29526616
    [TBL] [Abstract][Full Text] [Related]  

  • 35. Gene replacement therapy provides benefit in an adult mouse model of Leigh syndrome.
    Reynaud-Dulaurier R; Benegiamo G; Marrocco E; Al-Tannir R; Surace EM; Auwerx J; Decressac M
    Brain; 2020 Jun; 143(6):1686-1696. PubMed ID: 32413099
    [TBL] [Abstract][Full Text] [Related]  

  • 36. 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]  

  • 37. Early-age Ndufs4 knockout mice are an inappropriate animal model of Leigh syndrome.
    Finsterer J
    Radiol Phys Technol; 2019 Jun; 12(2):230-231. PubMed ID: 30937725
    [No Abstract]   [Full Text] [Related]  

  • 38. 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]  

  • 39. Low-dose rapamycin extends lifespan in a mouse model of mtDNA depletion syndrome.
    Siegmund SE; Yang H; Sharma R; Javors M; Skinner O; Mootha V; Hirano M; Schon EA
    Hum Mol Genet; 2017 Dec; 26(23):4588-4605. PubMed ID: 28973153
    [TBL] [Abstract][Full Text] [Related]  

  • 40. 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]  

    [Previous]   [Next]    [New Search]
    of 12.