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Complex V deficiency

MedGen UID:
452448
Concept ID:
C0342779
Disease or Syndrome
Synonyms: Complex 5 mitochondrial respiratory chain deficiency; Mitochondrial complex V deficiency
 
Related genes: ATPAF2, TMEM70, ATP5F1E

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Emmanuel IA, Olotu FA, Agoni C, Soliman MES
Curr Pharm Biotechnol 2020;21(14):1551-1566. doi: 10.2174/1389201021666200628152246. PMID: 32598251

Recent clinical studies

Etiology

Lines MA, Cuillerier A, Chakraborty P, Naas T, Duque Lasio ML, Michaud J, Pileggi C, Harper ME, Burelle Y, Toler TL, Sondheimer N, Crawford HP, Millan F, Geraghty MT
Eur J Hum Genet 2021 Nov;29(11):1719-1724. Epub 2021 Sep 6 doi: 10.1038/s41431-021-00956-0. PMID: 34483339Free PMC Article
Staretz-Chacham O, Wormser O, Manor E, Birk OS, Ferreira CR
Am J Med Genet A 2019 Jul;179(7):1293-1298. Epub 2019 Apr 4 doi: 10.1002/ajmg.a.61138. PMID: 30950220Free PMC Article
Shahrour MA, Staretz-Chacham O, Dayan D, Stephen J, Weech A, Damseh N, Pri Chen H, Edvardson S, Mazaheri S, Saada A; NISC Intramural Sequencing, Hershkovitz E, Shaag A, Huizing M, Abu-Libdeh B, Gahl WA, Azem A, Anikster Y, Vilboux T, Elpeleg O, Malicdan MC
Clin Genet 2017 May;91(5):690-696. Epub 2016 Oct 12 doi: 10.1111/cge.12855. PMID: 27573165Free PMC Article
Wortmann SB, Rodenburg RJ, Jonckheere A, de Vries MC, Huizing M, Heldt K, van den Heuvel LP, Wendel U, Kluijtmans LA, Engelke UF, Wevers RA, Smeitink JA, Morava E
Brain 2009 Jan;132(Pt 1):136-46. Epub 2008 Nov 16 doi: 10.1093/brain/awn296. PMID: 19015156
De Vivo DC
Brain Dev 1993 Jan-Feb;15(1):1-22. doi: 10.1016/0387-7604(93)90002-p. PMID: 8338207

Diagnosis

Staretz-Chacham O, Wormser O, Manor E, Birk OS, Ferreira CR
Am J Med Genet A 2019 Jul;179(7):1293-1298. Epub 2019 Apr 4 doi: 10.1002/ajmg.a.61138. PMID: 30950220Free PMC Article
Cohen I, Staretz-Chacham O, Wormser O, Perez Y, Saada A, Kadir R, Birk OS
Am J Med Genet A 2018 Feb;176(2):330-336. Epub 2017 Dec 11 doi: 10.1002/ajmg.a.38574. PMID: 29226520
Jackson CB, Hahn D, Schröter B, Richter U, Battersby BJ, Schmitt-Mechelke T, Marttinen P, Nuoffer JM, Schaller A
Eur J Med Genet 2017 Jun;60(6):345-351. Epub 2017 Apr 13 doi: 10.1016/j.ejmg.2017.04.006. PMID: 28412374
Jonckheere AI, Renkema GH, Bras M, van den Heuvel LP, Hoischen A, Gilissen C, Nabuurs SB, Huynen MA, de Vries MC, Smeitink JA, Rodenburg RJ
Brain 2013 May;136(Pt 5):1544-54. Epub 2013 Apr 18 doi: 10.1093/brain/awt086. PMID: 23599390
Ma YY, Zhang XL, Wu TF, Liu YP, Wang Q, Zhang Y, Song JQ, Wang YJ, Yang YL
J Child Neurol 2011 Aug;26(8):974-9. Epub 2011 May 3 doi: 10.1177/0883073811399905. PMID: 21540367

Prognosis

Lines MA, Cuillerier A, Chakraborty P, Naas T, Duque Lasio ML, Michaud J, Pileggi C, Harper ME, Burelle Y, Toler TL, Sondheimer N, Crawford HP, Millan F, Geraghty MT
Eur J Hum Genet 2021 Nov;29(11):1719-1724. Epub 2021 Sep 6 doi: 10.1038/s41431-021-00956-0. PMID: 34483339Free PMC Article
Shahrour MA, Staretz-Chacham O, Dayan D, Stephen J, Weech A, Damseh N, Pri Chen H, Edvardson S, Mazaheri S, Saada A; NISC Intramural Sequencing, Hershkovitz E, Shaag A, Huizing M, Abu-Libdeh B, Gahl WA, Azem A, Anikster Y, Vilboux T, Elpeleg O, Malicdan MC
Clin Genet 2017 May;91(5):690-696. Epub 2016 Oct 12 doi: 10.1111/cge.12855. PMID: 27573165Free PMC Article
Jonckheere AI, Huigsloot M, Lammens M, Jansen J, van den Heuvel LP, Spiekerkoetter U, von Kleist-Retzow JC, Forkink M, Koopman WJ, Szklarczyk R, Huynen MA, Fransen JA, Smeitink JA, Rodenburg RJ
Mitochondrion 2011 Nov;11(6):954-63. Epub 2011 Sep 14 doi: 10.1016/j.mito.2011.08.012. PMID: 21945727
De Meirleir L, Seneca S, Lissens W, De Clercq I, Eyskens F, Gerlo E, Smet J, Van Coster R
J Med Genet 2004 Feb;41(2):120-4. doi: 10.1136/jmg.2003.012047. PMID: 14757859Free PMC Article

Clinical prediction guides

Lines MA, Cuillerier A, Chakraborty P, Naas T, Duque Lasio ML, Michaud J, Pileggi C, Harper ME, Burelle Y, Toler TL, Sondheimer N, Crawford HP, Millan F, Geraghty MT
Eur J Hum Genet 2021 Nov;29(11):1719-1724. Epub 2021 Sep 6 doi: 10.1038/s41431-021-00956-0. PMID: 34483339Free PMC Article
Emmanuel IA, Olotu FA, Agoni C, Soliman MES
Curr Pharm Biotechnol 2020;21(14):1551-1566. doi: 10.2174/1389201021666200628152246. PMID: 32598251
Jonckheere AI, Renkema GH, Bras M, van den Heuvel LP, Hoischen A, Gilissen C, Nabuurs SB, Huynen MA, de Vries MC, Smeitink JA, Rodenburg RJ
Brain 2013 May;136(Pt 5):1544-54. Epub 2013 Apr 18 doi: 10.1093/brain/awt086. PMID: 23599390
Jonckheere AI, Huigsloot M, Lammens M, Jansen J, van den Heuvel LP, Spiekerkoetter U, von Kleist-Retzow JC, Forkink M, Koopman WJ, Szklarczyk R, Huynen MA, Fransen JA, Smeitink JA, Rodenburg RJ
Mitochondrion 2011 Nov;11(6):954-63. Epub 2011 Sep 14 doi: 10.1016/j.mito.2011.08.012. PMID: 21945727
Geromel V, Kadhom N, Cebalos-Picot I, Ouari O, Polidori A, Munnich A, Rötig A, Rustin P
Hum Mol Genet 2001 May 15;10(11):1221-8. doi: 10.1093/hmg/10.11.1221. PMID: 11371515

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