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Leigh syndrome due to mitochondrial complex IV deficiency

MedGen UID:
338082
Concept ID:
C1850599
Disease or Syndrome
Synonym: Leigh syndrome due to COX IV deficiency

Authors:
Shamima Rahman  |  David Thorburn   view full author information

Professional guidelines

PubMed

Honzik T, Tesarova M, Magner M, Mayr J, Jesina P, Vesela K, Wenchich L, Szentivanyi K, Hansikova H, Sperl W, Zeman J
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Gropman AL
Curr Neurol Neurosci Rep 2001 Mar;1(2):185-94. doi: 10.1007/s11910-001-0015-9. PMID: 11898515

Recent clinical studies

Etiology

Diaz F
Biochim Biophys Acta 2010 Jan;1802(1):100-10. Epub 2009 Aug 11 doi: 10.1016/j.bbadis.2009.07.013. PMID: 19682572
Filosto M, Tomelleri G, Tonin P, Scarpelli M, Vattemi G, Rizzuto N, Padovani A, Simonati A
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Robinson BH
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Di Donato S
J Inherit Metab Dis 2000 May;23(3):247-63. doi: 10.1023/a:1005684029429. PMID: 10863941
Zeviani M, Corona P, Nijtmans L, Tiranti V
Ital J Neurol Sci 1999 Dec;20(6):401-8. doi: 10.1007/s100720050059. PMID: 10937860

Diagnosis

Pesenti F, Doucet E, Morin C, Falcao M
Ophthalmic Genet 2018 Dec;39(6):725-727. Epub 2018 Nov 14 doi: 10.1080/13816810.2018.1546407. PMID: 30426811
Gropman AL
Curr Neurol Neurosci Rep 2001 Mar;1(2):185-94. doi: 10.1007/s11910-001-0015-9. PMID: 11898515
Shoubridge EA
Am J Med Genet 2001 Spring;106(1):46-52. doi: 10.1002/ajmg.1378. PMID: 11579424
Makino M, Horai S, Goto Y, Nonaka I
J Hum Genet 2000;45(2):69-75. doi: 10.1007/s100380050014. PMID: 10721666
Rahman S, Blok RB, Dahl HH, Danks DM, Kirby DM, Chow CW, Christodoulou J, Thorburn DR
Ann Neurol 1996 Mar;39(3):343-51. doi: 10.1002/ana.410390311. PMID: 8602753

Therapy

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 26;5:8035. doi: 10.1038/srep08035. PMID: 25620325Free PMC Article
Komaki H, Nishigaki Y, Fuku N, Hosoya H, Murayama K, Ohtake A, Goto Y, Wakamoto H, Koga Y, Tanaka M
Biochim Biophys Acta 2010 Mar;1800(3):313-5. Epub 2009 Jul 17 doi: 10.1016/j.bbagen.2009.07.008. PMID: 19616603
Goldenberg PC, Steiner RD, Merkens LS, Dunaway T, Egan RA, Zimmerman EA, Nesbit G, Robinson B, Kennaway NG
Neurology 2003 Mar 11;60(5):865-8. doi: 10.1212/01.wnl.0000049460.72439.7f. PMID: 12629249
Nonaka I, Koga Y, Kikuchi A, Goto Y
Acta Neuropathol 1991;82(4):286-94. doi: 10.1007/BF00308814. PMID: 1662001
Paulson DJ, Hoganson GE, Traxler J, Sufit R, Peters H, Shug AL
Biochem Med Metab Biol 1988 Feb;39(1):40-7. doi: 10.1016/0885-4505(88)90056-4. PMID: 2833295

Prognosis

Kotecha S, Kairamkonda V
BMJ Case Rep 2019 Jul 15;12(7) doi: 10.1136/bcr-2019-229668. PMID: 31308188Free PMC Article
Aulbert W, Weigt-Usinger K, Thiels C, Köhler C, Vorgerd M, Schreiner A, Hoffjan S, Rothoeft T, Wortmann SB, Heyer CM, Podskarbi T, Lücke T
Neuropediatrics 2014 Dec;45(6):346-53. Epub 2014 Aug 11 doi: 10.1055/s-0034-1383823. PMID: 25111564
Lee IC, El-Hattab AW, Wang J, Li FY, Weng SW, Craigen WJ, Wong LJ
Hum Mutat 2012 Aug;33(8):1192-200. Epub 2012 Apr 30 doi: 10.1002/humu.22095. PMID: 22488715
Farina L, Chiapparini L, Uziel G, Bugiani M, Zeviani M, Savoiardo M
AJNR Am J Neuroradiol 2002 Aug;23(7):1095-100. PMID: 12169463Free PMC Article
Makino M, Horai S, Goto Y, Nonaka I
J Hum Genet 2000;45(2):69-75. doi: 10.1007/s100380050014. PMID: 10721666

Clinical prediction guides

Mani S, Rao SN, Kranthi Kumar MV
J Neurol Sci 2019 Sep 15;404:91-100. Epub 2019 Jul 10 doi: 10.1016/j.jns.2019.07.007. PMID: 31352295
Sasarman F, Nishimura T, Antonicka H, Weraarpachai W, Shoubridge EA; LSFC Consortium
Hum Mol Genet 2015 Jan 15;24(2):480-91. Epub 2014 Sep 11 doi: 10.1093/hmg/ddu468. PMID: 25214534Free PMC Article
Lee IC, El-Hattab AW, Wang J, Li FY, Weng SW, Craigen WJ, Wong LJ
Hum Mutat 2012 Aug;33(8):1192-200. Epub 2012 Apr 30 doi: 10.1002/humu.22095. PMID: 22488715
Virgilio R, Ronchi D, Bordoni A, Fassone E, Bonato S, Donadoni C, Torgano G, Moggio M, Corti S, Bresolin N, Comi GP
J Neurol Sci 2009 Jun 15;281(1-2):85-92. Epub 2009 Mar 10 doi: 10.1016/j.jns.2009.01.025. PMID: 19278689
Di Donato S
J Inherit Metab Dis 2000 May;23(3):247-63. doi: 10.1023/a:1005684029429. PMID: 10863941

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