Literature DB >> 20802310

Nicolaides-Baraitser syndrome: two new cases with autism spectrum disorder.

Simone Gana1, Michela Panizzon, Daniela Fongaro, Angelo Selicorni, Luigi Memo, Valeria Scandurra, Chiara Vannucci, Marta Bigozzi, Maria Rosaria Scordo.   

Abstract

Nicolaides-Baraitser syndrome is a rare clinical condition characterized by mental retardation with impairment of expressive language, short stature, microcephaly, sparse hair, typical facial dysmorphisms, and interphalangeal joint swellings. To date 24 cases have been reported, most of them being sporadic. The genetic background of Nicolaides-Baraitser syndrome is unclear in terms of cause and mode of inheritance, one of the more probable explanations is de novo mutation of a dominant gene. Some reported patients presented autistic features, although in none of these patients was the diagnosis of autism spectrum disorder formally made. We describe two unrelated patients with clinical features suggesting Nicolaides-Baraitser syndrome and, in addition, autism spectrum disorder is defined by the presence of the three cardinal core features: qualitative impairments in social, communicative, and behavioral development.

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Year:  2011        PMID: 20802310     DOI: 10.1097/MCD.0b013e32833edaa9

Source DB:  PubMed          Journal:  Clin Dysmorphol        ISSN: 0962-8827            Impact factor:   0.816


  5 in total

1.  Camk2a-Cre-mediated conditional deletion of chromatin remodeler Brg1 causes perinatal hydrocephalus.

Authors:  Mou Cao; Jiang I Wu
Journal:  Neurosci Lett       Date:  2015-04-27       Impact factor: 3.046

2.  In-Frame Deletion and Missense Mutations of the C-Terminal Helicase Domain of SMARCA2 in Three Patients with Nicolaides-Baraitser Syndrome.

Authors:  D Wolff; S Endele; S Azzarello-Burri; J Hoyer; M Zweier; I Schanze; B Schmitt; A Rauch; A Reis; C Zweier
Journal:  Mol Syndromol       Date:  2012-03-16

3.  Autism-Associated Chromatin Regulator Brg1/SmarcA4 Is Required for Synapse Development and Myocyte Enhancer Factor 2-Mediated Synapse Remodeling.

Authors:  Zilai Zhang; Mou Cao; Chia-Wei Chang; Cindy Wang; Xuanming Shi; Xiaoming Zhan; Shari G Birnbaum; Ilya Bezprozvanny; Kimberly M Huber; Jiang I Wu
Journal:  Mol Cell Biol       Date:  2015-10-12       Impact factor: 4.272

4.  A SMARCA2 Mutation in the First Case Report of Nicolaides-Baraitser Syndrome in Latin America: Genotype-Phenotype Correlation.

Authors:  Ana Isabel Sánchez; Jorge Armando Rojas
Journal:  Case Rep Genet       Date:  2017-08-29

5.  Heat shock alters the expression of schizophrenia and autism candidate genes in an induced pluripotent stem cell model of the human telencephalon.

Authors:  Mingyan Lin; Dejian Zhao; Anastasia Hrabovsky; Erika Pedrosa; Deyou Zheng; Herbert M Lachman
Journal:  PLoS One       Date:  2014-04-15       Impact factor: 3.240

  5 in total

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