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Chromosome 2p16.3 deletion syndrome

MedGen UID:
814824
Concept ID:
C3808494
Disease or Syndrome
Synonym: CHROMOSOME 2p16.3 DELETION SYNDROME
 
Gene (location): NRXN1 (2p16.3)
 
Monarch Initiative: MONDO:0013696
OMIM®: 614332

Definition

Deletions at 2p16.3 involving exons of NRXN1 are associated with susceptibility to autism, schizophrenia (SCZD17), developmental delay, intellectual disability, and dysmorphic features. The phenotype is highly variable and shows incomplete penetrance (summary by Dabell et al., 2013). For a phenotypic description and a discussion of genetic heterogeneity of schizophrenia, see 181500. [from OMIM]

Recent clinical studies

Etiology

Castronovo P, Baccarin M, Ricciardello A, Picinelli C, Tomaiuolo P, Cucinotta F, Frittoli M, Lintas C, Sacco R, Persico AM
Clin Genet 2020 Jan;97(1):125-137. Epub 2019 May 7 doi: 10.1111/cge.13537. PMID: 30873608
Soueid J, Kourtian S, Makhoul NJ, Makoukji J, Haddad S, Ghanem SS, Kobeissy F, Boustany RM
Sci Rep 2016 Jan 8;6:19088. doi: 10.1038/srep19088. PMID: 26742492Free PMC Article
Vassos E, Collier DA, Holden S, Patch C, Rujescu D, St Clair D, Lewis CM
Hum Mol Genet 2010 Sep 1;19(17):3477-81. Epub 2010 Jun 29 doi: 10.1093/hmg/ddq259. PMID: 20587603

Diagnosis

Alfieri P, Scibelli F, Sinibaldi L, Valeri G, Caciolo C, Novello RL, Novelli A, Digilio MC, Tartaglia M, Vicari S
Genes Brain Behav 2020 Sep;19(7):e12687. Epub 2020 Aug 26 doi: 10.1111/gbb.12687. PMID: 32658356
Zhang H, Xi Q, Liu X, Yue F, Zhang H, Sun M, Liu R
Biomed Res Int 2020;2020:1761738. Epub 2020 May 27 doi: 10.1155/2020/1761738. PMID: 32566663Free PMC Article
Salo-Mullen EE, Lynn PB, Wang L, Walsh M, Gopalan A, Shia J, Tran C, Man FY, McBride S, Schattner M, Zhang L, Weiser MR, Stadler ZK
Fam Cancer 2018 Jan;17(1):71-77. doi: 10.1007/s10689-017-0006-x. PMID: 28555354Free PMC Article
Soueid J, Kourtian S, Makhoul NJ, Makoukji J, Haddad S, Ghanem SS, Kobeissy F, Boustany RM
Sci Rep 2016 Jan 8;6:19088. doi: 10.1038/srep19088. PMID: 26742492Free PMC Article
Wallis M, Tsurusaki Y, Burgess T, Borzi P, Matsumoto N, Miyake N, True D, Patel C
Am J Med Genet A 2016 Mar;170(3):717-24. Epub 2015 Nov 21 doi: 10.1002/ajmg.a.37478. PMID: 26590955

Prognosis

Zhang H, Xi Q, Liu X, Yue F, Zhang H, Sun M, Liu R
Biomed Res Int 2020;2020:1761738. Epub 2020 May 27 doi: 10.1155/2020/1761738. PMID: 32566663Free PMC Article
Castronovo P, Baccarin M, Ricciardello A, Picinelli C, Tomaiuolo P, Cucinotta F, Frittoli M, Lintas C, Sacco R, Persico AM
Clin Genet 2020 Jan;97(1):125-137. Epub 2019 May 7 doi: 10.1111/cge.13537. PMID: 30873608
Wallis M, Tsurusaki Y, Burgess T, Borzi P, Matsumoto N, Miyake N, True D, Patel C
Am J Med Genet A 2016 Mar;170(3):717-24. Epub 2015 Nov 21 doi: 10.1002/ajmg.a.37478. PMID: 26590955
Vassos E, Collier DA, Holden S, Patch C, Rujescu D, St Clair D, Lewis CM
Hum Mol Genet 2010 Sep 1;19(17):3477-81. Epub 2010 Jun 29 doi: 10.1093/hmg/ddq259. PMID: 20587603

Clinical prediction guides

Alfieri P, Scibelli F, Sinibaldi L, Valeri G, Caciolo C, Novello RL, Novelli A, Digilio MC, Tartaglia M, Vicari S
Genes Brain Behav 2020 Sep;19(7):e12687. Epub 2020 Aug 26 doi: 10.1111/gbb.12687. PMID: 32658356
Zhang H, Xi Q, Liu X, Yue F, Zhang H, Sun M, Liu R
Biomed Res Int 2020;2020:1761738. Epub 2020 May 27 doi: 10.1155/2020/1761738. PMID: 32566663Free PMC Article
Castronovo P, Baccarin M, Ricciardello A, Picinelli C, Tomaiuolo P, Cucinotta F, Frittoli M, Lintas C, Sacco R, Persico AM
Clin Genet 2020 Jan;97(1):125-137. Epub 2019 May 7 doi: 10.1111/cge.13537. PMID: 30873608
Wallis M, Tsurusaki Y, Burgess T, Borzi P, Matsumoto N, Miyake N, True D, Patel C
Am J Med Genet A 2016 Mar;170(3):717-24. Epub 2015 Nov 21 doi: 10.1002/ajmg.a.37478. PMID: 26590955
Vassos E, Collier DA, Holden S, Patch C, Rujescu D, St Clair D, Lewis CM
Hum Mol Genet 2010 Sep 1;19(17):3477-81. Epub 2010 Jun 29 doi: 10.1093/hmg/ddq259. PMID: 20587603

Recent systematic reviews

Castronovo P, Baccarin M, Ricciardello A, Picinelli C, Tomaiuolo P, Cucinotta F, Frittoli M, Lintas C, Sacco R, Persico AM
Clin Genet 2020 Jan;97(1):125-137. Epub 2019 May 7 doi: 10.1111/cge.13537. PMID: 30873608

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