Literature DB >> 26997944

Xp22.33p22.12 Duplication in a Patient with Intellectual Disability and Dysmorphic Facial Features.

Carla Lintas1, Chiara Picinelli2, Ignazio S Piras2, Roberto Sacco1, Stefano Gabriele3, Magda Verdecchia4, Antonio M Persico5.   

Abstract

A novel 19.98-Mb duplication in chromosome Xp22.33p22.12 was detected by array CGH in a 30-year-old man affected by intellectual disability, congenital hypotonia and dysmorphic features. The duplication encompasses more than 100 known genes. Many of these genes (such as neuroligin 4, cyclin-dependent kinase like 5, and others) have already correlated with X-linked intellectual disability and/or neurodevelopmental disorders. Due to the high number of potentially pathogenic genes involved in the reported duplication, we cannot correlate the clinical phenotype to a single gene. Indeed, we suggest that the resulting clinical phenotype may have arisen from the overexpression and consequent perturbation of fine gene dosage.

Entities:  

Keywords:  Duplication Xp22.33p22.12; Dysmorphic facial features; Intellectual disability

Year:  2016        PMID: 26997944      PMCID: PMC4772714          DOI: 10.1159/000443232

Source DB:  PubMed          Journal:  Mol Syndromol        ISSN: 1661-8769


  14 in total

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Review 2.  Genome-wide expression studies in autism spectrum disorder, Rett syndrome, and Down syndrome.

Authors:  Carla Lintas; Roberto Sacco; Antonio M Persico
Journal:  Neurobiol Dis       Date:  2010-12-02       Impact factor: 5.996

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4.  The 4P-syndrome. Case description and literature review.

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5.  Range of genetic mutations associated with severe non-syndromic sporadic intellectual disability: an exome sequencing study.

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Journal:  Lancet       Date:  2012-09-27       Impact factor: 79.321

6.  An Xp21.3p11.4 duplication observed in a boy with intellectual deficiency and speech delay and his asymptomatic mother.

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Review 7.  X-linked mental retardation.

Authors:  H-Hilger Ropers; Ben C J Hamel
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Journal:  Proc Natl Acad Sci U S A       Date:  1993-12-15       Impact factor: 11.205

10.  STRING v9.1: protein-protein interaction networks, with increased coverage and integration.

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Journal:  Nucleic Acids Res       Date:  2012-11-29       Impact factor: 16.971

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  3 in total

1.  Genetic Counselling Pitfall: Co-Occurrence of an 11.8-Mb Xp22 Duplication and an Xp21.2 Duplication Disrupting IL1RAPL1.

Authors:  Nicolas Chatron; Lucie Thibault; James Lespinasse; Audrey Labalme; Caroline Schluth-Bolard; Marianne Till; Patrick Edery; Renaud Touraine; Vincent des Portes; Gaetan Lesca; Damien Sanlaville
Journal:  Mol Syndromol       Date:  2017-09-07

2.  Identification of Novel Congenital Heart Disease Candidate Genes Using Chromosome Microarray.

Authors:  Enas Shanshen; Janine Rosenberg; Andrew H Van Bergen
Journal:  Pediatr Cardiol       Date:  2017-10-09       Impact factor: 1.655

3.  Whole genome sequencing identifies a duplicated region encompassing Xq13.2q13.3 in a large Iranian family with intellectual disability.

Authors:  Sepideh Mehvari; Farzaneh Larti; Hao Hu; Zohreh Fattahi; Maryam Beheshtian; Seyedeh Sedigheh Abedini; Sanaz Arzhangi; Hans-Hilger Ropers; Vera M Kalscheuer; Daniel Auld; Kimia Kahrizi; Yasser Riazalhosseini; Hossein Najmabadi
Journal:  Mol Genet Genomic Med       Date:  2020-07-26       Impact factor: 2.183

  3 in total

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