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Skewed maternal X inactivation

MedGen UID:
868462
Concept ID:
C4022856
Cell or Molecular Dysfunction
HPO: HP:0012546

Definition

A deviation from equal (50%) inactivation of each parental X chromosome in maternal cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSkewed maternal X inactivation

Professional guidelines

PubMed

Radic CP, Rossetti LC, Abelleyro MM, Tetzlaff T, Candela M, Neme D, Sciuccati G, Bonduel M, Medina-Acosta E, Larripa IB, de Tezanos Pinto M, De Brasi CD
J Thromb Haemost 2015 Apr;13(4):530-9. Epub 2015 Mar 14 doi: 10.1111/jth.12854. PMID: 25611311
Wengler GS, Parolini O, Fiorini M, Mella P, Smith H, Ugazio AG, Notarangelo LD
Life Sci 1997;61(14):1405-11. doi: 10.1016/s0024-3205(97)00686-3. PMID: 9335230
Allen RC, Nachtman RG, Rosenblatt HM, Belmont JW
Am J Hum Genet 1994 Jan;54(1):25-35. PMID: 7506482Free PMC Article

Recent clinical studies

Etiology

Juchniewicz P, Kloska A, Portalska K, Jakóbkiewicz-Banecka J, Węgrzyn G, Liss J, Głodek P, Tukaj S, Piotrowska E
Chromosome Res 2023 Jan 25;31(1):4. doi: 10.1007/s10577-023-09717-9. PMID: 36695960Free PMC Article
Roberts AL, Morea A, Amar A, Zito A, El-Sayed Moustafa JS, Tomlinson M, Bowyer RCE, Zhang X, Christiansen C, Costeira R, Steves CJ, Mangino M, Bell JT, Wong CCY, Vyse TJ, Small KS
Elife 2022 Nov 22;11 doi: 10.7554/eLife.78263. PMID: 36412098Free PMC Article
Duan X, Yang S, Zhang H, Wu J, Zhang Y, Ji D, Tie L, Boerkoel CF
J Dent Res 2019 Jul;98(8):870-878. Epub 2019 Jun 11 doi: 10.1177/0022034519854973. PMID: 31185186
Mengel-From J, Thinggaard M, Christiansen L, Vaupel JW, Orstavik KH, Christensen K
Eur J Hum Genet 2012 Mar;20(3):361-4. Epub 2011 Dec 7 doi: 10.1038/ejhg.2011.215. PMID: 22146940Free PMC Article
Uz E, Loubiere LS, Gadi VK, Ozbalkan Z, Stewart J, Nelson JL, Ozcelik T
Clin Rev Allergy Immunol 2008 Jun;34(3):352-5. doi: 10.1007/s12016-007-8044-z. PMID: 18157513Free PMC Article

Diagnosis

Fadra N, Schultz-Rogers LE, Chanana P, Cousin MA, Macke EL, Ferrer A, Pinto E Vairo F, Olson RJ, Oliver GR, Mulvihill LA, Jenkinson G, Klee EW
BMC Genomics 2024 Apr 16;25(1):371. doi: 10.1186/s12864-024-10240-2. PMID: 38627676Free PMC Article
Batlle-Masó L, Rivière JG, Franco-Jarava C, Martín-Nalda A, Garcia-Prat M, Parra-Martínez A, Aguiló-Cucurull A, Castells N, Martinez-Gallo M, Soler-Palacín P, Colobran R
J Clin Immunol 2023 Nov;43(8):1953-1963. Epub 2023 Aug 19 doi: 10.1007/s10875-023-01556-x. PMID: 37597073
Giovenino C, Trajkova S, Pavinato L, Cardaropoli S, Pullano V, Ferrero E, Sukarova-Angelovska E, Carestiato S, Salmin P, Rinninella A, Battaglia A, Bertoli L, Fadda A, Palermo F, Carli D, Mussa A, Dimartino P, Bruselles A, Froukh T, Mandrile G, Pasini B, De Rubeis S, Buxbaum JD, Pippucci T, Tartaglia M, Rossato M, Delledonne M, Ferrero GB, Brusco A
Eur J Hum Genet 2023 Nov;31(11):1228-1236. Epub 2023 Mar 6 doi: 10.1038/s41431-023-01324-w. PMID: 36879111Free PMC Article
Roberts AL, Morea A, Amar A, Zito A, El-Sayed Moustafa JS, Tomlinson M, Bowyer RCE, Zhang X, Christiansen C, Costeira R, Steves CJ, Mangino M, Bell JT, Wong CCY, Vyse TJ, Small KS
Elife 2022 Nov 22;11 doi: 10.7554/eLife.78263. PMID: 36412098Free PMC Article
Cho RY, Peñaherrera MS, Du Souich C, Huang L, Mwenifumbo J, Nelson TN, Elliott AM, Adam S; CAUSES Study, Eydoux P, Yang GX, Chijiwa C, Van Allen MI, Friedman JM, Robinson WP, Lehman A
Am J Med Genet A 2020 Mar;182(3):498-503. Epub 2019 Dec 16 doi: 10.1002/ajmg.a.61451. PMID: 31840929

Therapy

Giovenino C, Trajkova S, Pavinato L, Cardaropoli S, Pullano V, Ferrero E, Sukarova-Angelovska E, Carestiato S, Salmin P, Rinninella A, Battaglia A, Bertoli L, Fadda A, Palermo F, Carli D, Mussa A, Dimartino P, Bruselles A, Froukh T, Mandrile G, Pasini B, De Rubeis S, Buxbaum JD, Pippucci T, Tartaglia M, Rossato M, Delledonne M, Ferrero GB, Brusco A
Eur J Hum Genet 2023 Nov;31(11):1228-1236. Epub 2023 Mar 6 doi: 10.1038/s41431-023-01324-w. PMID: 36879111Free PMC Article
Juchniewicz P, Kloska A, Portalska K, Jakóbkiewicz-Banecka J, Węgrzyn G, Liss J, Głodek P, Tukaj S, Piotrowska E
Chromosome Res 2023 Jan 25;31(1):4. doi: 10.1007/s10577-023-09717-9. PMID: 36695960Free PMC Article
Roberts AL, Morea A, Amar A, Zito A, El-Sayed Moustafa JS, Tomlinson M, Bowyer RCE, Zhang X, Christiansen C, Costeira R, Steves CJ, Mangino M, Bell JT, Wong CCY, Vyse TJ, Small KS
Elife 2022 Nov 22;11 doi: 10.7554/eLife.78263. PMID: 36412098Free PMC Article
Shvetsova E, Sofronova A, Monajemi R, Gagalova K, Draisma HHM, White SJ, Santen GWE, Chuva de Sousa Lopes SM, Heijmans BT, van Meurs J, Jansen R, Franke L, Kiełbasa SM, den Dunnen JT, 't Hoen PAC; BIOS consortium; GoNL consortium
Eur J Hum Genet 2019 Mar;27(3):455-465. Epub 2018 Dec 14 doi: 10.1038/s41431-018-0291-3. PMID: 30552425Free PMC Article
Migeon BR
Cytogenet Cell Genet 1998;80(1-4):142-8. doi: 10.1159/000014971. PMID: 9678349

Prognosis

Roberts AL, Morea A, Amar A, Zito A, El-Sayed Moustafa JS, Tomlinson M, Bowyer RCE, Zhang X, Christiansen C, Costeira R, Steves CJ, Mangino M, Bell JT, Wong CCY, Vyse TJ, Small KS
Elife 2022 Nov 22;11 doi: 10.7554/eLife.78263. PMID: 36412098Free PMC Article
Tønne E, Holdhus R, Stansberg C, Stray-Pedersen A, Petersen K, Brunner HG, Gilissen C, Hoischen A, Prescott T, Steen VM, Fiskerstrand T
Eur J Hum Genet 2015 Dec;23(12):1652-6. Epub 2015 Mar 4 doi: 10.1038/ejhg.2015.30. PMID: 25735484Free PMC Article
Mengel-From J, Thinggaard M, Christiansen L, Vaupel JW, Orstavik KH, Christensen K
Eur J Hum Genet 2012 Mar;20(3):361-4. Epub 2011 Dec 7 doi: 10.1038/ejhg.2011.215. PMID: 22146940Free PMC Article
Hogge WA, Prosen TL, Lanasa MC, Huber HA, Reeves MF
Am J Obstet Gynecol 2007 Apr;196(4):384.e1-6; discussion 384.e6-8. doi: 10.1016/j.ajog.2006.12.012. PMID: 17403428
Renault NK, Dyack S, Dobson MJ, Costa T, Lam WL, Greer WL
Eur J Hum Genet 2007 Jun;15(6):628-37. Epub 2007 Mar 7 doi: 10.1038/sj.ejhg.5201799. PMID: 17342157

Clinical prediction guides

Juchniewicz P, Kloska A, Portalska K, Jakóbkiewicz-Banecka J, Węgrzyn G, Liss J, Głodek P, Tukaj S, Piotrowska E
Chromosome Res 2023 Jan 25;31(1):4. doi: 10.1007/s10577-023-09717-9. PMID: 36695960Free PMC Article
Delanne J, Lecat M, Blackburn PR, Klee EW, Stumpel CTRM, Stegmann S, Stevens SJC, Nava C, Heron D, Keren B, Mahida S, Naidu S, Babovic-Vuksanovic D, Herkert JC, Torring PM, Kibæk M, De Bie I, Pfundt R, Hendriks YMC, Ousager LB, Bend R, Warren H, Skinner SA, Lyons MJ, Pöe C, Chevarin M, Jouan T, Garde A, Thomas Q, Kuentz P, Tisserant E, Duffourd Y, Philippe C, Faivre L, Thauvin-Robinet C
Eur J Med Genet 2023 Jan;66(1):104670. Epub 2022 Nov 19 doi: 10.1016/j.ejmg.2022.104670. PMID: 36414205
Roberts AL, Morea A, Amar A, Zito A, El-Sayed Moustafa JS, Tomlinson M, Bowyer RCE, Zhang X, Christiansen C, Costeira R, Steves CJ, Mangino M, Bell JT, Wong CCY, Vyse TJ, Small KS
Elife 2022 Nov 22;11 doi: 10.7554/eLife.78263. PMID: 36412098Free PMC Article
Fang X, Butler KM, Abidi F, Gass J, Beisang A, Feyma T, Ryther RC, Standridge S, Heydemann P, Jones M, Haas R, Lieberman DN, Marsh ED, Benke TA, Skinner S, Neul JL, Percy AK, Friez MJ, Caylor RC
Mol Genet Genomic Med 2022 May;10(5):e1917. Epub 2022 Mar 23 doi: 10.1002/mgg3.1917. PMID: 35318820Free PMC Article
Shvetsova E, Sofronova A, Monajemi R, Gagalova K, Draisma HHM, White SJ, Santen GWE, Chuva de Sousa Lopes SM, Heijmans BT, van Meurs J, Jansen R, Franke L, Kiełbasa SM, den Dunnen JT, 't Hoen PAC; BIOS consortium; GoNL consortium
Eur J Hum Genet 2019 Mar;27(3):455-465. Epub 2018 Dec 14 doi: 10.1038/s41431-018-0291-3. PMID: 30552425Free PMC Article

Recent systematic reviews

Blyth U, Craciunas L, Hudson G, Choudhary M
Hum Reprod Update 2021 Aug 20;27(5):866-884. doi: 10.1093/humupd/dmab010. PMID: 33969392
Blue NR, Page JM, Silver RM
Semin Perinatol 2019 Mar;43(2):66-73. Epub 2018 Dec 19 doi: 10.1053/j.semperi.2018.12.002. PMID: 30638594Free PMC Article
Wang Z, Wang P, Wang X, He X, Wang Z, Xu D, Hu J, Wang B
Metabolism 2013 Sep;62(9):1227-38. Epub 2013 Jul 11 doi: 10.1016/j.metabol.2013.03.003. PMID: 23850180

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