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Cytochrome c oxidase I deficiency

MedGen UID:
865039
Concept ID:
C4016602
Finding
Synonym: CYTOCHROME c OXIDASE I DEFICIENCY
 
OMIM®: 516030

Professional guidelines

PubMed

Pfeffer G, Chinnery PF
Ann Med 2013 Feb;45(1):4-16. Epub 2011 Aug 25 doi: 10.3109/07853890.2011.605389. PMID: 21867371Free PMC Article
Bourgeron T, Chrétien D, Rötig A, Munnich A, Rustin P
Prenat Diagn 1992 Jun;12(6):548-9. doi: 10.1002/pd.1970120614. PMID: 1325051
Ruitenbeek W, Sengers R, Albani M, Trijbels F, Janssen A, van Diggelen O, Bakkeren J
N Engl J Med 1988 Oct 20;319(16):1095. doi: 10.1056/NEJM198810203191616. PMID: 2845269

Recent clinical studies

Etiology

Čunátová K, Fernández-Vizarra E
J Inherit Metab Dis 2024 Nov;47(6):1278-1291. Epub 2024 Jul 25 doi: 10.1002/jimd.12751. PMID: 39053894Free PMC Article
Pfeffer G, Chinnery PF
Ann Med 2013 Feb;45(1):4-16. Epub 2011 Aug 25 doi: 10.3109/07853890.2011.605389. PMID: 21867371Free PMC Article
Schapira AH
Curr Opin Neurol 2000 Oct;13(5):527-32. doi: 10.1097/00019052-200010000-00004. PMID: 11073358
Poulton J
Bioessays 1992 Nov;14(11):763-8. doi: 10.1002/bies.950141108. PMID: 1365889
DiMauro S, Bonilla E, Zeviani M, Servidei S, DeVivo DC, Schon EA
J Inherit Metab Dis 1987;10 Suppl 1:113-28. doi: 10.1007/BF01812852. PMID: 2824920

Diagnosis

Zhang Z, Yu R, Shi Q, Wu ZJ, Li Q, Mu J, Chen B, Shi J, Ni R, Wu L, Li Q, Fu J, Li R, Sun X, Wang J, He L, Kuang Y, Sang Q, Wang L
Proc Natl Acad Sci U S A 2024 Nov 5;121(45):e2406174121. Epub 2024 Oct 29 doi: 10.1073/pnas.2406174121. PMID: 39471219Free PMC Article
Rak M, Bénit P, Chrétien D, Bouchereau J, Schiff M, El-Khoury R, Tzagoloff A, Rustin P
Clin Sci (Lond) 2016 Mar;130(6):393-407. doi: 10.1042/CS20150707. PMID: 26846578Free PMC Article
Chen Z, Zhong C
Prog Neurobiol 2013 Sep;108:21-43. Epub 2013 Jul 11 doi: 10.1016/j.pneurobio.2013.06.004. PMID: 23850509
Pfeffer G, Chinnery PF
Ann Med 2013 Feb;45(1):4-16. Epub 2011 Aug 25 doi: 10.3109/07853890.2011.605389. PMID: 21867371Free PMC Article
DiMauro S, Moraes CT
Arch Neurol 1993 Nov;50(11):1197-208. doi: 10.1001/archneur.1993.00540110075008. PMID: 8215979

Therapy

Pfeffer G, Chinnery PF
Ann Med 2013 Feb;45(1):4-16. Epub 2011 Aug 25 doi: 10.3109/07853890.2011.605389. PMID: 21867371Free PMC Article
Schapira AH
Curr Opin Neurol 2000 Oct;13(5):527-32. doi: 10.1097/00019052-200010000-00004. PMID: 11073358
Batocchi AP, Evoli A, Servidei S, Palmisani MT, Apollo F, Tonali P
Neurology 1995 Feb;45(2):382-3. doi: 10.1212/wnl.45.2.382. PMID: 7854543
Müller-Höcker J
Brain Pathol 1992 Apr;2(2):149-58. doi: 10.1111/j.1750-3639.1992.tb00683.x. PMID: 1341956
Coltman CA Jr
JAMA 1969 Jan 20;207(3):513-6. PMID: 4303073

Prognosis

Bonora E, Chakrabarty S, Kellaris G, Tsutsumi M, Bianco F, Bergamini C, Ullah F, Isidori F, Liparulo I, Diquigiovanni C, Masin L, Rizzardi N, Cratere MG, Boschetti E, Papa V, Maresca A, Cenacchi G, Casadio R, Martelli P, Matera I, Ceccherini I, Fato R, Raiola G, Arrigo S, Signa S, Sementa AR, Severino M, Striano P, Fiorillo C, Goto T, Uchino S, Oyazato Y, Nakamura H, Mishra SK, Yeh YS, Kato T, Nozu K, Tanboon J, Morioka I, Nishino I, Toda T, Goto YI, Ohtake A, Kosaki K, Yamaguchi Y, Nonaka I, Iijima K, Mimaki M, Kurahashi H, Raams A, MacInnes A, Alders M, Engelen M, Linthorst G, de Koning T, den Dunnen W, Dijkstra G, van Spaendonck K, van Gent DC, Aronica EM, Picco P, Carelli V, Seri M, Katsanis N, Duijkers FAM, Taniguchi-Ikeda M, De Giorgio R
Brain 2021 Jun 22;144(5):1451-1466. doi: 10.1093/brain/awab056. PMID: 33855352
Rak M, Bénit P, Chrétien D, Bouchereau J, Schiff M, El-Khoury R, Tzagoloff A, Rustin P
Clin Sci (Lond) 2016 Mar;130(6):393-407. doi: 10.1042/CS20150707. PMID: 26846578Free PMC Article
Cifelli PM, Hargreaves I, Grünewald S
J Inherit Metab Dis 2002 Sep;25(5):411-2. doi: 10.1023/a:1020112119716. PMID: 12408191
Percival SS
Am J Clin Nutr 1998 May;67(5 Suppl):1064S-1068S. doi: 10.1093/ajcn/67.5.1064S. PMID: 9587153
Garavaglia B, Colamaria V, Carrara F, Tonin P, Rimoldi M, Uziel G
J Inherit Metab Dis 1994;17(3):301-3. doi: 10.1007/BF00711813. PMID: 7807937

Clinical prediction guides

Choong CJ, Okuno T, Ikenaka K, Baba K, Hayakawa H, Koike M, Yokota M, Doi J, Kakuda K, Takeuchi T, Kuma A, Nakamura S, Nagai Y, Nagano S, Yoshimori T, Mochizuki H
Autophagy 2021 Oct;17(10):2962-2974. Epub 2020 Dec 10 doi: 10.1080/15548627.2020.1848130. PMID: 33218272Free PMC Article
Rak M, Bénit P, Chrétien D, Bouchereau J, Schiff M, El-Khoury R, Tzagoloff A, Rustin P
Clin Sci (Lond) 2016 Mar;130(6):393-407. doi: 10.1042/CS20150707. PMID: 26846578Free PMC Article
Lalonde R, Strazielle C
Cerebellum 2003;2(4):300-9. doi: 10.1080/14734220310017456. PMID: 14964689
Mazat JP, Rossignol R, Malgat M, Rocher C, Faustin B, Letellier T
Biochim Biophys Acta 2001 Mar 1;1504(1):20-30. doi: 10.1016/s0005-2728(00)00236-x. PMID: 11239482
Percival SS
Am J Clin Nutr 1998 May;67(5 Suppl):1064S-1068S. doi: 10.1093/ajcn/67.5.1064S. PMID: 9587153

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