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MED12-related intellectual disability syndrome

MedGen UID:
988374
Concept ID:
CN305246
Disease or Syndrome
Synonyms: MED12 X-linked syndromic intellectual disability; X-linked syndromic intellectual disability caused by mutation in MED12
 
Monarch Initiative: MONDO:0100000

Definition

An X-linked syndromic intellectual disability that that includes subtypes of the heterogeneous, eponymously named Lujan-Fryns syndrome, X-linked Ohdo syndrome, and Optiz-Kaveggia/ FG syndrome, which is caused by mutations in the gene MED12. The common and most penetrant phenotype shared amongst these disease entities is intellectual disability, with dysgenesis or agenesis of the corpus callosum, blepharophimosis, and marfanoid habitus having variable phenotypic expressivity. [from MONDO]

Recent clinical studies

Etiology

Shah A, Bapna M, Al-Saif H, Li R, Couser NL
Ophthalmic Genet 2022 Feb;43(1):126-129. Epub 2021 Oct 20 doi: 10.1080/13816810.2021.1989601. PMID: 34670449
Charzewska A, Maiwald R, Kahrizi K, Oehl-Jaschkowitz B, Dufke A, Lemke JR, Enders H, Najmabadi H, Tzschach A, Hachmann W, Jensen C, Bienek M, Poznański J, Nawara M, Chilarska T, Obersztyn E, Hoffman-Zacharska D, Gos M, Bal J, Kalscheuer VM
Clin Genet 2018 Nov;94(5):450-456. Epub 2018 Aug 9 doi: 10.1111/cge.13412. PMID: 30006928

Diagnosis

Maia N, Ibarluzea N, Misra-Isrie M, Koboldt DC, Marques I, Soares G, Santos R, Marcelis CLM, Keski-Filppula R, Guitart M, Gabau Vila E, Lehman A, Hickey S, Mori M, Terhal P, Valenzuela I, Lasa-Aranzasti A, Cueto-González AM, Chhouk BH, Yeh RC, Neil JE, Abu-Libde B, Kleefstra T, Elting MW, Császár A, Kárteszi J, Bessenyei B, van Bokhoven H, Jorge P, van Hagen JM, de Brouwer APM
Am J Med Genet A 2023 Jan;191(1):135-143. Epub 2022 Oct 22 doi: 10.1002/ajmg.a.63004. PMID: 36271811Free PMC Article
Prescott TE, Kulseth MA, Heimdal KR, Stadheim B, Hopp E, Gambin T, Coban Akdemir ZH, Jhangiani SN, Muzny DM, Gibbs RA, Lupski JR, Stray-Pedersen A
Eur J Med Genet 2016 Aug;59(8):367-72. Epub 2016 Jun 7 doi: 10.1016/j.ejmg.2016.06.001. PMID: 27286923
Graham JM Jr, Schwartz CE
Am J Med Genet A 2013 Nov;161A(11):2734-40. Epub 2013 Oct 10 doi: 10.1002/ajmg.a.36183. PMID: 24123922Free PMC Article

Therapy

Maia N, Ibarluzea N, Misra-Isrie M, Koboldt DC, Marques I, Soares G, Santos R, Marcelis CLM, Keski-Filppula R, Guitart M, Gabau Vila E, Lehman A, Hickey S, Mori M, Terhal P, Valenzuela I, Lasa-Aranzasti A, Cueto-González AM, Chhouk BH, Yeh RC, Neil JE, Abu-Libde B, Kleefstra T, Elting MW, Császár A, Kárteszi J, Bessenyei B, van Bokhoven H, Jorge P, van Hagen JM, de Brouwer APM
Am J Med Genet A 2023 Jan;191(1):135-143. Epub 2022 Oct 22 doi: 10.1002/ajmg.a.63004. PMID: 36271811Free PMC Article
Zhi X, Pu L, Wu B, Cui Y, Yu C, Dong Y, Li D, Cai C
Clin Chim Acta 2022 Jul 1;532:137-144. Epub 2022 Jun 8 doi: 10.1016/j.cca.2022.05.023. PMID: 35690084

Prognosis

Charzewska A, Maiwald R, Kahrizi K, Oehl-Jaschkowitz B, Dufke A, Lemke JR, Enders H, Najmabadi H, Tzschach A, Hachmann W, Jensen C, Bienek M, Poznański J, Nawara M, Chilarska T, Obersztyn E, Hoffman-Zacharska D, Gos M, Bal J, Kalscheuer VM
Clin Genet 2018 Nov;94(5):450-456. Epub 2018 Aug 9 doi: 10.1111/cge.13412. PMID: 30006928
Prontera P, Ottaviani V, Rogaia D, Isidori I, Mencarelli A, Malerba N, Cocciadiferro D, Rolph P, Stangoni G, Vulto-van Silfhout A, Merla G
Am J Med Genet A 2016 Sep;170(9):2377-82. Epub 2016 Jun 17 doi: 10.1002/ajmg.a.37805. PMID: 27312080

Clinical prediction guides

Charzewska A, Maiwald R, Kahrizi K, Oehl-Jaschkowitz B, Dufke A, Lemke JR, Enders H, Najmabadi H, Tzschach A, Hachmann W, Jensen C, Bienek M, Poznański J, Nawara M, Chilarska T, Obersztyn E, Hoffman-Zacharska D, Gos M, Bal J, Kalscheuer VM
Clin Genet 2018 Nov;94(5):450-456. Epub 2018 Aug 9 doi: 10.1111/cge.13412. PMID: 30006928
Prontera P, Ottaviani V, Rogaia D, Isidori I, Mencarelli A, Malerba N, Cocciadiferro D, Rolph P, Stangoni G, Vulto-van Silfhout A, Merla G
Am J Med Genet A 2016 Sep;170(9):2377-82. Epub 2016 Jun 17 doi: 10.1002/ajmg.a.37805. PMID: 27312080

Recent systematic reviews

Shah A, Bapna M, Al-Saif H, Li R, Couser NL
Ophthalmic Genet 2022 Feb;43(1):126-129. Epub 2021 Oct 20 doi: 10.1080/13816810.2021.1989601. PMID: 34670449

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