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Mitochondrial disorder due to a defect in assembly or maturation of the respiratory chain complexes

MedGen UID:
1843278
Concept ID:
C5681078
Disease or Syndrome
Orphanet: ORPHA309136

Professional guidelines

PubMed

Wu T, He F, Xiao N, Han Y, Yang L, Peng J
Pediatr Neurol 2022 Jul;132:11-18. Epub 2022 Apr 30 doi: 10.1016/j.pediatrneurol.2022.04.013. PMID: 35598585
Rahman S
Epilepsy Behav 2019 Dec;101(Pt B):106546. Epub 2019 Oct 31 doi: 10.1016/j.yebeh.2019.106546. PMID: 31677997
Schon EA, DiMauro S
Novartis Found Symp 2007;287:214-25; discussion 226-33. doi: 10.1002/9780470725207.ch15. PMID: 18074641

Recent clinical studies

Etiology

Deneubourg C, Ramm M, Smith LJ, Baron O, Singh K, Byrne SC, Duchen MR, Gautel M, Eskelinen EL, Fanto M, Jungbluth H
Autophagy 2022 Mar;18(3):496-517. Epub 2021 Aug 19 doi: 10.1080/15548627.2021.1943177. PMID: 34130600Free PMC Article
Miles EA, Childs CE, Calder PC
Nutrients 2021 Jan 16;13(1) doi: 10.3390/nu13010247. PMID: 33467123Free PMC Article
Rahman S
Epilepsy Behav 2019 Dec;101(Pt B):106546. Epub 2019 Oct 31 doi: 10.1016/j.yebeh.2019.106546. PMID: 31677997
Bragoszewski P, Turek M, Chacinska A
Open Biol 2017 Apr;7(4) doi: 10.1098/rsob.170007. PMID: 28446709Free PMC Article
Schapira AH
Curr Opin Neurol 2000 Oct;13(5):527-32. doi: 10.1097/00019052-200010000-00004. PMID: 11073358

Diagnosis

Rahman S
Handb Clin Neurol 2023;194:43-63. doi: 10.1016/B978-0-12-821751-1.00015-4. PMID: 36813320
Deneubourg C, Ramm M, Smith LJ, Baron O, Singh K, Byrne SC, Duchen MR, Gautel M, Eskelinen EL, Fanto M, Jungbluth H
Autophagy 2022 Mar;18(3):496-517. Epub 2021 Aug 19 doi: 10.1080/15548627.2021.1943177. PMID: 34130600Free PMC Article
Wang T, Liu H, Itoh K, Oh S, Zhao L, Murata D, Sesaki H, Hartung T, Na CH, Wang J
Cell Metab 2021 Mar 2;33(3):531-546.e9. Epub 2021 Feb 4 doi: 10.1016/j.cmet.2021.01.005. PMID: 33545050Free PMC Article
El-Hattab AW, Scaglia F
Cell Calcium 2016 Sep;60(3):199-206. Epub 2016 Mar 4 doi: 10.1016/j.ceca.2016.03.003. PMID: 26996063
Zeviani M, Tiranti V, Piantadosi C
Medicine (Baltimore) 1998 Jan;77(1):59-72. doi: 10.1097/00005792-199801000-00006. PMID: 9465864

Therapy

Turpin R, Liu R, Munne PM, Peura A, Rannikko JH, Philips G, Boeckx B, Salmelin N, Hurskainen E, Suleymanova I, Aung J, Vuorinen EM, Lehtinen L, Mutka M, Kovanen PE, Niinikoski L, Meretoja TJ, Mattson J, Mustjoki S, Saavalainen P, Goga A, Lambrechts D, Pouwels J, Hollmén M, Klefström J
J Immunother Cancer 2024 Apr 11;12(4) doi: 10.1136/jitc-2023-008053. PMID: 38604809Free PMC Article
Sabharwal A, Campbell JM, Schwab TL, WareJoncas Z, Wishman MD, Ata H, Liu W, Ichino N, Hunter DE, Bergren JD, Urban MD, Urban RM, Holmberg SR, Kar B, Cook A, Ding Y, Xu X, Clark KJ, Ekker SC
Genes (Basel) 2022 Jul 23;13(8) doi: 10.3390/genes13081317. PMID: 35893052Free PMC Article
Kaustio M, Nayebzadeh N, Hinttala R, Tapiainen T, Åström P, Mamia K, Pernaa N, Lehtonen J, Glumoff V, Rahikkala E, Honkila M, Olsén P, Hassinen A, Polso M, Al Sukaiti N, Al Shekaili J, Al Kindi M, Al Hashmi N, Almusa H, Bulanova D, Haapaniemi E, Chen P, Suo-Palosaari M, Vieira P, Tuominen H, Kokkonen H, Al Macki N, Al Habsi H, Löppönen T, Rantala H, Pietiäinen V, Zhang SY, Renko M, Hautala T, Al Farsi T, Uusimaa J, Saarela J
J Allergy Clin Immunol 2021 Aug;148(2):599-611. Epub 2021 Mar 1 doi: 10.1016/j.jaci.2020.12.656. PMID: 33662367
Rahman S
Epilepsy Behav 2019 Dec;101(Pt B):106546. Epub 2019 Oct 31 doi: 10.1016/j.yebeh.2019.106546. PMID: 31677997
Schapira AH
Curr Opin Neurol 2000 Oct;13(5):527-32. doi: 10.1097/00019052-200010000-00004. PMID: 11073358

Prognosis

van der Ven AT, Cabrera-Orefice A, Wente I, Feichtinger RG, Tsiakas K, Weiss D, Bierhals T, Scholle L, Prokisch H, Kopajtich R, Santer R, Mayr JA, Hempel M, Wittig I
Mol Genet Metab 2023 Nov;140(3):107675. Epub 2023 Aug 4 doi: 10.1016/j.ymgme.2023.107675. PMID: 37572574
Sabharwal A, Campbell JM, Schwab TL, WareJoncas Z, Wishman MD, Ata H, Liu W, Ichino N, Hunter DE, Bergren JD, Urban MD, Urban RM, Holmberg SR, Kar B, Cook A, Ding Y, Xu X, Clark KJ, Ekker SC
Genes (Basel) 2022 Jul 23;13(8) doi: 10.3390/genes13081317. PMID: 35893052Free PMC Article
Deneubourg C, Ramm M, Smith LJ, Baron O, Singh K, Byrne SC, Duchen MR, Gautel M, Eskelinen EL, Fanto M, Jungbluth H
Autophagy 2022 Mar;18(3):496-517. Epub 2021 Aug 19 doi: 10.1080/15548627.2021.1943177. PMID: 34130600Free PMC Article
El-Hattab AW, Scaglia F
Cell Calcium 2016 Sep;60(3):199-206. Epub 2016 Mar 4 doi: 10.1016/j.ceca.2016.03.003. PMID: 26996063
Mayr JA, Haack TB, Freisinger P, Karall D, Makowski C, Koch J, Feichtinger RG, Zimmermann FA, Rolinski B, Ahting U, Meitinger T, Prokisch H, Sperl W
J Inherit Metab Dis 2015 Jul;38(4):629-40. Epub 2015 Mar 17 doi: 10.1007/s10545-015-9831-y. PMID: 25778941Free PMC Article

Clinical prediction guides

Wang J, Qiu Y, Yang L, Wang J, He J, Tang C, Yang Z, Hong W, Yang B, He Q, Weng Q
Autophagy 2024 Dec;20(12):2677-2696. Epub 2024 Aug 4 doi: 10.1080/15548627.2024.2384348. PMID: 39099169Free PMC Article
Ast T, Itoh Y, Sadre S, McCoy JG, Namkoong G, Wengrod JC, Chicherin I, Joshi PR, Kamenski P, Suess DLM, Amunts A, Mootha VK
Mol Cell 2024 Jan 18;84(2):359-374.e8. Epub 2024 Jan 9 doi: 10.1016/j.molcel.2023.12.016. PMID: 38199006Free PMC Article
Doni D, Cavion F, Bortolus M, Baschiera E, Muccioli S, Tombesi G, d'Ettorre F, Ottaviani D, Marchesan E, Leanza L, Greggio E, Ziviani E, Russo A, Bellin M, Sartori G, Carbonera D, Salviati L, Costantini P
Cell Death Dis 2023 Dec 8;14(12):805. doi: 10.1038/s41419-023-06320-y. PMID: 38062036Free PMC Article
Guhathakurta S, Erdogdu NU, Hoffmann JJ, Grzadzielewska I, Schendzielorz A, Seyfferth J, Mårtensson CU, Corrado M, Karoutas A, Warscheid B, Pfanner N, Becker T, Akhtar A
Nat Metab 2023 Nov;5(11):1931-1952. Epub 2023 Oct 9 doi: 10.1038/s42255-023-00904-w. PMID: 37813994Free PMC Article
Gemmer M, Chaillet ML, van Loenhout J, Cuevas Arenas R, Vismpas D, Gröllers-Mulderij M, Koh FA, Albanese P, Scheltema RA, Howes SC, Kotecha A, Fedry J, Förster F
Nature 2023 Feb;614(7946):160-167. Epub 2023 Jan 25 doi: 10.1038/s41586-022-05638-5. PMID: 36697828Free PMC Article

Recent systematic reviews

Tauchmannová K, Pecinová A, Houštěk J, Mráček T
Physiol Res 2024 Aug 31;73(Suppl 1):S243-S278. Epub 2024 Jul 17 doi: 10.33549/physiolres.935407. PMID: 39016153Free PMC Article
Fassone E, Rahman S
J Med Genet 2012 Sep;49(9):578-90. doi: 10.1136/jmedgenet-2012-101159. PMID: 22972949

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