نتایج جستجو برای: autosomal recessive non syndromic hearing loss arnshl

تعداد نتایج: 1782155  

Journal: :Journal of medical genetics 2002
S H Blanton C Y Liang M W Cai A Pandya L L Du B Landa S Mummalanni K S Li Z Y Chen X N Qin Y F Liu T Balkany W E Nance X Z Liu

We have studied 36 subjects in a large multigenerational Chinese family that is segregating for an autosomal dominant adult onset form of progressive non-syndromic hearing loss. All affected subjects had bilateral sensorineural hearing loss involving all frequencies with some significant gender differences in initial presentation. After excluding linkage to known loci for non-syndromic deafness...

Journal: :European journal of medical genetics 2014
Érika L Freitas Jeanne Oiticica Amanda G Silva Roseli S M Bittar Carla Rosenberg Regina C Mingroni-Netto

In 20% of cases, hereditary non-syndromic hearing loss has an autosomal dominant inheritance (ADNSHL). To date, more than 50 loci for ADNSHL have been mapped to different chromosomal regions. In order to verify whether genomic alterations contribute to the hearing loss etiology and to search for novel deafness candidate loci, we investigated probands from families with ADNSHL by oligonucleotide...

Marjan Mojtabavi Naeini Morteza Hashemzadeh Chaleshtori Sadeq Vallian Boroujeni

Background & Aims: SLC26A4 gene mutations are the second identifiable genetic cause of autosomal recessive nonsyndromic hearing loss (ARNSHL) after GJB2 mutations and are currently investigated in molecular diagnosis.In databases, several potential STR markers related to this region have been introduced. In this investigation, the characteristics and informativeness of D7S2425 CA repeat STR mar...

Journal: :Journal of medical genetics 2005
N J Leonard A L Krol S Bleoo M J Somerville

M utations in connexin 26 (GJB2, Cx26) cause autosomal recessive and occasionally dominant non-syndromic sensorineural hearing loss (SNHL). Cx26 mutations have also been identified in SNHL with dermatological features of autosomal dominant diffuse palmoplantar hyperkeratosis (DPPK). We describe a girl with bilateral sloping sensorineural hearing loss, striate palmoplantar hyperkeratosis (SPPK),...

2017
Jinsei Jung Joon Suk Lee Kyeong Jee Cho Seyoung Yu Joo-Heon Yoon Heon Yung Gee Jae Young Choi

Discriminating between inherited and non-inherited sporadic hearing loss is challenging. Here, we attempted to delineate genetic inheritance in simplex cases of severe-to-profound congenital hearing loss in Korean children. Variations in SLC26A4 and GJB2 in 28 children with bilateral severe-to-profound non-syndromic hearing loss (NSHL) without familial history were analyzed using Sanger sequenc...

ژورنال: :genetics in the 3rd millennium 0
مرجان مجتبوی نائینی marjan mojtabavi naeini صادق ولیان بروجنی sadeq vallian borujeni مرتضی هاشم زاده چالشتری morteza hashemzadeh chaleshtori

جهش های ژن slc26a4 پس از ژن gjb2 مهم ترین عامل ژنتیکی ایجاد کننده ناشنوایی غیر سندرمی با وراثت اتوزومی مغلوب (autosomal recessive non-syndromic hearing loss, arnshl) هستند که امروزه در تشخیص های مولکولی مورد بررسی قرار می گیرند. در پایگاه داده ها تعداد زیادی از مارکرهای str مرتبط با این ناحیه معرفی شده است. در این مطالعه، خصوصیات و اطلاع دهندگی مارکر d7s2420 با توالی های تکراری ca، که در ناحیه ...

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