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Combined oxidative phosphorylation deficiency

MedGen UID:
1626645
Concept ID:
C4540031
Finding
Synonym: combined oxidative phosphorylation deficiency
 
Related genes: MICOS13, EARS2, MTFMT, SFXN4, SLC25A26, MARS2, MTRFR, PNPT1, GFM1, GTPBP3, TARS2, NARS2, CARS2, MRPS34, MRPL44, ELAC2, TRMT5, AARS2, VARS2, LYRM4, MRPS22, RMND1, TRMT10C, TRIT1, MRPS2, MRPS7, MRPS16, TXN2, MTO1, MRPL3, FARS2, TSFM, AIFM1, TUFM, MIPEP, C1QBP, ATP5F1A
 
Monarch Initiative: MONDO:0000732
OMIM® Phenotypic series: PS609060

Definition

A mitochondrial oxidative phosphorylation disorder in which multiple mitochondrial respiratory chain complexes. [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Patnaik MM, Tefferi A
Am J Hematol 2021 Mar 1;96(3):379-394. Epub 2021 Jan 28 doi: 10.1002/ajh.26090. PMID: 33428785
Ahmed ST, Craven L, Russell OM, Turnbull DM, Vincent AE
Neurotherapeutics 2018 Oct;15(4):943-953. doi: 10.1007/s13311-018-00674-4. PMID: 30406383Free PMC Article
Stroes ES, Thompson PD, Corsini A, Vladutiu GD, Raal FJ, Ray KK, Roden M, Stein E, Tokgözoğlu L, Nordestgaard BG, Bruckert E, De Backer G, Krauss RM, Laufs U, Santos RD, Hegele RA, Hovingh GK, Leiter LA, Mach F, März W, Newman CB, Wiklund O, Jacobson TA, Catapano AL, Chapman MJ, Ginsberg HN; European Atherosclerosis Society Consensus Panel
Eur Heart J 2015 May 1;36(17):1012-22. Epub 2015 Feb 18 doi: 10.1093/eurheartj/ehv043. PMID: 25694464Free PMC Article

Recent clinical studies

Etiology

Aleksic D, Jankovic MG, Todorovic S, Kovacevic M, Borkovic M
Turk J Pediatr 2023;65(6):1018-1024. doi: 10.24953/turkjped.2022.1082. PMID: 38204316
Angelova P, Velchev V, Stoyanov N, Atemin S, Todorov T, Tourtourikov I, Mitev V, Todorova A
Am J Med Genet A 2023 Jul;191(7):1804-1813. Epub 2023 Apr 7 doi: 10.1002/ajmg.a.63205. PMID: 37029485
Ni M, Black LF, Pan C, Vu H, Pei J, Ko B, Cai L, Solmonson A, Yang C, Nugent KM, Grishin NV, Xing C, Roeder E, DeBerardinis RJ
J Inherit Metab Dis 2021 Jul;44(4):949-960. Epub 2021 Apr 27 doi: 10.1002/jimd.12387. PMID: 33855712Free PMC Article
O'Byrne JJ, Tarailo-Graovac M, Ghani A, Champion M, Deshpande C, Dursun A, Ozgul RK, Freisinger P, Garber I, Haack TB, Horvath R, Barić I, Husain RA, Kluijtmans LAJ, Kotzaeridou U, Morris AA, Ross CJ, Santra S, Smeitink J, Tarnopolsky M, Wortmann SB, Mayr JA, Brunner-Krainz M, Prokisch H, Wasserman WW, Wevers RA, Engelke UF, Rodenburg RJ, Ting TW, McFarland R, Taylor RW, Salvarinova R, van Karnebeek CDM
Mol Genet Metab 2018 Jan;123(1):28-42. Epub 2017 Nov 15 doi: 10.1016/j.ymgme.2017.11.003. PMID: 29331171Free PMC Article
van Diemen CC, Kerstjens-Frederikse WS, Bergman KA, de Koning TJ, Sikkema-Raddatz B, van der Velde JK, Abbott KM, Herkert JC, Löhner K, Rump P, Meems-Veldhuis MT, Neerincx PBT, Jongbloed JDH, van Ravenswaaij-Arts CM, Swertz MA, Sinke RJ, van Langen IM, Wijmenga C
Pediatrics 2017 Oct;140(4) doi: 10.1542/peds.2016-2854. PMID: 28939701

Diagnosis

Dong Q, Yin X, Fan S, Zhong S, Yang W, Chen K, Wang Q, Ma X, Mahlatsi RL, Yang Y, Lyu J, Fang H, Wang Y
Orphanet J Rare Dis 2024 Aug 21;19(1):305. doi: 10.1186/s13023-024-03310-x. PMID: 39169373Free PMC Article
Ji Y, Wang S, Cheng Y, Fang L, Zhao J, Gao L, Xu C
Gene 2021 Dec 15;804:145891. Epub 2021 Aug 8 doi: 10.1016/j.gene.2021.145891. PMID: 34375635
Alsharhan H, Muraresku C, Ganetzky RD
Am J Med Genet A 2021 Aug;185(8):2519-2525. Epub 2021 May 19 doi: 10.1002/ajmg.a.62344. PMID: 34008913
van Diemen CC, Kerstjens-Frederikse WS, Bergman KA, de Koning TJ, Sikkema-Raddatz B, van der Velde JK, Abbott KM, Herkert JC, Löhner K, Rump P, Meems-Veldhuis MT, Neerincx PBT, Jongbloed JDH, van Ravenswaaij-Arts CM, Swertz MA, Sinke RJ, van Langen IM, Wijmenga C
Pediatrics 2017 Oct;140(4) doi: 10.1542/peds.2016-2854. PMID: 28939701
Alfadhel M, Nashabat M, Abu Ali Q, Hundallah K
Neurosciences (Riyadh) 2017 Jan;22(1):4-13. doi: 10.17712/nsj.2017.1.20160542. PMID: 28064324Free PMC Article

Therapy

van Diemen CC, Kerstjens-Frederikse WS, Bergman KA, de Koning TJ, Sikkema-Raddatz B, van der Velde JK, Abbott KM, Herkert JC, Löhner K, Rump P, Meems-Veldhuis MT, Neerincx PBT, Jongbloed JDH, van Ravenswaaij-Arts CM, Swertz MA, Sinke RJ, van Langen IM, Wijmenga C
Pediatrics 2017 Oct;140(4) doi: 10.1542/peds.2016-2854. PMID: 28939701
Raviglione F, Conte G, Ghezzi D, Parazzini C, Righini A, Vergaro R, Legati A, Spaccini L, Gasperini S, Garavaglia B, Mastrangelo M
Am J Med Genet A 2016 Nov;170(11):3004-3007. Epub 2016 Aug 23 doi: 10.1002/ajmg.a.37836. PMID: 27549011

Prognosis

Dong Q, Yin X, Fan S, Zhong S, Yang W, Chen K, Wang Q, Ma X, Mahlatsi RL, Yang Y, Lyu J, Fang H, Wang Y
Orphanet J Rare Dis 2024 Aug 21;19(1):305. doi: 10.1186/s13023-024-03310-x. PMID: 39169373Free PMC Article
Zhang X, Xiang F, Li D, Yang F, Yu S, Wang X
BMC Neurol 2024 Jan 2;24(1):15. doi: 10.1186/s12883-023-03480-4. PMID: 38166857Free PMC Article
Ji Y, Wang S, Cheng Y, Fang L, Zhao J, Gao L, Xu C
Gene 2021 Dec 15;804:145891. Epub 2021 Aug 8 doi: 10.1016/j.gene.2021.145891. PMID: 34375635
van Diemen CC, Kerstjens-Frederikse WS, Bergman KA, de Koning TJ, Sikkema-Raddatz B, van der Velde JK, Abbott KM, Herkert JC, Löhner K, Rump P, Meems-Veldhuis MT, Neerincx PBT, Jongbloed JDH, van Ravenswaaij-Arts CM, Swertz MA, Sinke RJ, van Langen IM, Wijmenga C
Pediatrics 2017 Oct;140(4) doi: 10.1542/peds.2016-2854. PMID: 28939701
Vernon HJ, McClellan R, Batista DA, Naidu S
Am J Med Genet A 2015 May;167A(5):1147-51. Epub 2015 Apr 6 doi: 10.1002/ajmg.a.36993. PMID: 25851414

Clinical prediction guides

Dong Q, Yin X, Fan S, Zhong S, Yang W, Chen K, Wang Q, Ma X, Mahlatsi RL, Yang Y, Lyu J, Fang H, Wang Y
Orphanet J Rare Dis 2024 Aug 21;19(1):305. doi: 10.1186/s13023-024-03310-x. PMID: 39169373Free PMC Article
Smith TB, Rea A, Thomas HB, Thompson K, Oláhová M, Maroofian R, Zamani M, He L, Sadeghian S, Galehdari H, Lotan NS, Gilboa T, Herman KC, McCorvie TJ, Yue WW, Houlden H, Taylor RW, Newman WG, O'Keefe RT
Eur J Hum Genet 2023 Oct;31(10):1190-1194. Epub 2023 Aug 9 doi: 10.1038/s41431-023-01437-2. PMID: 37558808Free PMC Article
Angelova P, Velchev V, Stoyanov N, Atemin S, Todorov T, Tourtourikov I, Mitev V, Todorova A
Am J Med Genet A 2023 Jul;191(7):1804-1813. Epub 2023 Apr 7 doi: 10.1002/ajmg.a.63205. PMID: 37029485
Ji Y, Wang S, Cheng Y, Fang L, Zhao J, Gao L, Xu C
Gene 2021 Dec 15;804:145891. Epub 2021 Aug 8 doi: 10.1016/j.gene.2021.145891. PMID: 34375635
Vernon HJ, McClellan R, Batista DA, Naidu S
Am J Med Genet A 2015 May;167A(5):1147-51. Epub 2015 Apr 6 doi: 10.1002/ajmg.a.36993. PMID: 25851414

Recent systematic reviews

Zhang Q, Ouyang Q, Xiang J, Li H, Lv H, An Y
Genes (Basel) 2023 Feb 22;14(3) doi: 10.3390/genes14030552. PMID: 36980825Free PMC Article
He P, Wang Q, Hong X, Yuan H
Am J Med Genet A 2023 Jan;191(1):70-76. Epub 2022 Oct 11 doi: 10.1002/ajmg.a.62988. PMID: 36218002

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