نتایج جستجو برای: nonsyndromic

تعداد نتایج: 1750  

2011
Yajie Lu Dachun Dai Zhibin Chen Xin Cao Xingkuan Bu Qinjun Wei Guangqian Xing

Hearing loss is the most frequent sensory disorder involving a multitude of factors, and at least 50% of cases are due to genetic etiology. To further characterize the molecular etiology of hearing loss in the Chinese population, we recruited a total of 135 unrelated patients with nonsyndromic sensorineural hearing loss (NSHL) for mutational screening of GJB2, GJB3, GJB6, SLC26A4, SLC26A5 IVS2-...

Journal: :Science 2012
Stéphane J Baudouin Julien Gaudias Stefan Gerharz Laetitia Hatstatt Kuikui Zhou Pradeep Punnakkal Kenji F Tanaka Will Spooren Rene Hen Chris I De Zeeuw Kaspar Vogt Peter Scheiffele

The genetic heterogeneity of autism poses a major challenge for identifying mechanism-based treatments. A number of rare mutations are associated with autism, and it is unclear whether these result in common neuronal alterations. Monogenic syndromes, such as fragile X, include autism as one of their multifaceted symptoms and have revealed specific defects in synaptic plasticity. We discovered a...

2018
Haiqiong Shang Denise Yan Naeimeh Tayebi Kolsoum Saeidi Afsaneh Sahebalzamani Yong Feng Susan Blanton Xuezhong Liu

Hearing loss (HL) is a common sensory disorder in humans with high genetic heterogeneity. To date, over 145 loci have been identified to cause nonsyndromic deafness. Furthermore, there are countless families unsuitable for the conventional linkage analysis. In the present study, we used a custom capture panel (MiamiOtoGenes) to target sequence 180 deafness-associated genes in 5 GJB2 negative de...

Journal: :Investigative ophthalmology & visual science 2001
S Khaliq A Hameed M Ismail K Anwar B Leroy A M Payne S S Bhattacharya S Q Mehdi

PURPOSE To map the disease locus in a six-generation, consanguineous Pakistani family affected by nonsyndromic autosomal recessive persistent hyperplastic primary vitreous (arPHPV). All affected individuals had peripheral anterior synechiae and corneal opacities with variable degrees of cataract and a retrolenticular white mass behind the lens. METHODS Genomic DNA from family members was type...

Journal: :American journal of medical genetics. Part A 2011
Niloofar Bazazzadegan Abraham M Sheffield Masoomeh Sobhani Kimia Kahrizi Nicole C Meyer Guy Van Camp Nele Hilgert Seyedeh Sedigheh Abedini Farkhondeh Habibi Ahmad Daneshi Carla Nishimura Matthew R Avenarius Mohammad Farhadi Richard J H Smith Hossein Najmabadi

Mutations in GJB2, encoding connexin 26 (Cx26), cause both autosomal dominant and autosomal recessive nonsyndromic hearing loss (ARNSHL) at the DFNA3 and DFNB1 loci, respectively. Most of the over 100 described GJB2 mutations cause ARNSHL. Only a minority has been associated with autosomal dominant hearing loss. In this study, we present two families with autosomal dominant nonsyndromic hearing...

2016
Betti Giusti Stefano Nistri Elena Sticchi Rosina De Cario Rosanna Abbate Gian Franco Gensini Guglielmina Pepe

Thoracic aortic aneurysm/dissection (TAAD) is a potential lethal condition with a rising incidence. This condition may occur sporadically; nevertheless, it displays familial clustering in >20% of the cases. Family history confers a six- to twentyfold increased risk of TAAD and has to be considered in the identification and evaluation of patients needing an adequate clinical follow-up. Familial ...

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