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Stimulation of cholesterol biosynthesis in mitochondrial complex I-deficiency lowers reductive stress and improves motor function and survival in mice.
Schirris TJJ, Rossell S, de Haas R, Frambach SJCM, Hoogstraten CA, Renkema GH, Beyrath JD, Willems PHGM, Huynen MA, Smeitink JAM, Russel FGM, Notebaart RA. Schirris TJJ, et al. Among authors: de haas r. Biochim Biophys Acta Mol Basis Dis. 2021 Apr 1;1867(4):166062. doi: 10.1016/j.bbadis.2020.166062. Epub 2021 Jan 13. Biochim Biophys Acta Mol Basis Dis. 2021. PMID: 33385517 Free article.
NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4-/- mice and Leigh syndrome patients: A stabilizing role for NDUFAF2.
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. Adjobo-Hermans MJW, et al. Among authors: de haas r. Biochim Biophys Acta Bioenerg. 2020 Aug 1;1861(8):148213. doi: 10.1016/j.bbabio.2020.148213. Epub 2020 Apr 23. Biochim Biophys Acta Bioenerg. 2020. PMID: 32335026 Free article.
163 results