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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. van de Wal MAE, et al. Among authors: adjobo hermans mjw. Brain. 2022 Mar 29;145(1):45-63. doi: 10.1093/brain/awab426. Brain. 2022. PMID: 34849584 Free PMC article. Review.
Mitochondrial Morphofunction in Mammalian Cells.
Bulthuis EP, Adjobo-Hermans MJW, Willems PHGM, Koopman WJH. Bulthuis EP, et al. Among authors: adjobo hermans mjw. Antioxid Redox Signal. 2019 Jun 20;30(18):2066-2109. doi: 10.1089/ars.2018.7534. Epub 2018 Nov 29. Antioxid Redox Signal. 2019. PMID: 30266077 Free PMC article. Review.
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. 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.
The ketogenic diet as a therapeutic intervention strategy in mitochondrial disease.
Qu C, Keijer J, Adjobo-Hermans MJW, van de Wal M, Schirris T, van Karnebeek C, Pan Y, Koopman WJH. Qu C, et al. Among authors: adjobo hermans mjw. Int J Biochem Cell Biol. 2021 Sep;138:106050. doi: 10.1016/j.biocel.2021.106050. Epub 2021 Jul 21. Int J Biochem Cell Biol. 2021. PMID: 34298163 Free article. Review.
The decylTPP mitochondria-targeting moiety lowers electron transport chain supercomplex levels in primary human skin fibroblasts.
Bulthuis EP, Einer C, Distelmaier F, Groh L, van Emst-de Vries SE, van de Westerlo E, van de Wal M, Wagenaars J, Rodenburg RJ, Smeitink JAM, Riksen NP, Willems PHGM, Adjobo-Hermans MJW, Zischka H, Koopman WJH. Bulthuis EP, et al. Among authors: adjobo hermans mjw. Free Radic Biol Med. 2022 Aug 1;188:434-446. doi: 10.1016/j.freeradbiomed.2022.06.011. Epub 2022 Jun 17. Free Radic Biol Med. 2022. PMID: 35718301 Free article.
Stress-dependent macromolecular crowding in the mitochondrial matrix.
Bulthuis EP, Dieteren CEJ, Bergmans J, Berkhout J, Wagenaars JA, van de Westerlo EMA, Podhumljak E, Hink MA, Hesp LFB, Rosa HS, Malik AN, Lindert MK, Willems PHGM, Gardeniers HJGE, den Otter WK, Adjobo-Hermans MJW, Koopman WJH. Bulthuis EP, et al. Among authors: adjobo hermans mjw. EMBO J. 2023 Apr 3;42(7):e108533. doi: 10.15252/embj.2021108533. Epub 2023 Feb 24. EMBO J. 2023. PMID: 36825437 Free PMC article.
SMDT1 variants impair EMRE-mediated mitochondrial calcium uptake in patients with muscle involvement.
Bulthuis EP, Adjobo-Hermans MJW, de Potter B, Hoogstraten S, Wezendonk LHT, Tutakhel OAZ, Wintjes LT, van den Heuvel B, Willems PHGM, Kamsteeg EJ, Gozalbo MER, Sallevelt SCEH, Koudijs SM, Nicolai J, de Bie CI, Hoogendijk JE, Koopman WJH, Rodenburg RJ. Bulthuis EP, et al. Among authors: adjobo hermans mjw. Biochim Biophys Acta Mol Basis Dis. 2023 Dec;1869(8):166808. doi: 10.1016/j.bbadis.2023.166808. Epub 2023 Jul 16. Biochim Biophys Acta Mol Basis Dis. 2023. PMID: 37454773 Free article.
44 results