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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
J Inherit Metab Dis 2012 Sep;35(5):749-59. Epub 2012 Jan 10 doi: 10.1007/s10545-011-9440-3. PMID: 22231385
Darin N, Moslemi AR, Lebon S, Rustin P, Holme E, Oldfors A, Tulinius M
Neuropediatrics 2003 Dec;34(6):311-7. doi: 10.1055/s-2003-44670. PMID: 14681757
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

Kayani S, Daescu V, Dahshi H, Messahel S, Woleban K, Minassian BA, Ling Q, Gray SJ
Am J Med Genet A 2025 Apr;197(4):e63947. Epub 2024 Dec 5 doi: 10.1002/ajmg.a.63947. PMID: 39632678
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
Biosci Rep 2007 Jun;27(1-3):23-30. doi: 10.1007/s10540-007-9034-3. PMID: 17541738
Robinson BH
Pediatr Res 2000 Nov;48(5):581-5. doi: 10.1203/00006450-200011000-00004. PMID: 11044474
Di Donato S
J Inherit Metab Dis 2000 May;23(3):247-63. doi: 10.1023/a:1005684029429. PMID: 10863941

Diagnosis

Kayani S, Daescu V, Dahshi H, Messahel S, Woleban K, Minassian BA, Ling Q, Gray SJ
Am J Med Genet A 2025 Apr;197(4):e63947. Epub 2024 Dec 5 doi: 10.1002/ajmg.a.63947. PMID: 39632678
van Riesen AK, Antonicka H, Ohlenbusch A, Shoubridge EA, Wilichowski EK
Neuropediatrics 2006 Apr;37(2):88-94. doi: 10.1055/s-2006-924227. PMID: 16773507
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
Willis TA, Davidson J, Gray RG, Poulton K, Ramani P, Whitehouse W
Dev Med Child Neurol 2000 Jun;42(6):414-7. doi: 10.1017/s0012162200000761. PMID: 10875529

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

Kayani S, Daescu V, Dahshi H, Messahel S, Woleban K, Minassian BA, Ling Q, Gray SJ
Am J Med Genet A 2025 Apr;197(4):e63947. Epub 2024 Dec 5 doi: 10.1002/ajmg.a.63947. PMID: 39632678
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
Willis TA, Davidson J, Gray RG, Poulton K, Ramani P, Whitehouse W
Dev Med Child Neurol 2000 Jun;42(6):414-7. doi: 10.1017/s0012162200000761. PMID: 10875529
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

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
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
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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