نتایج جستجو برای: joubert syndrome

تعداد نتایج: 622071  

Journal: :Journal of Ophthalmic and Vision Research 2019

2016
Hee Gyung Kang Hyun Kyung Lee Yo Han Ahn Je-Gun Joung Jaeyong Nam Nayoung K D Kim Jung Min Ko Min Hyun Cho Jae Il Shin Joon Kim Hye Won Park Young Seo Park Il-Soo Ha Woo Yeong Chung Dae-Yeol Lee Su Young Kim Woong Yang Park Hae Il Cheong

Nephronophthisis-related ciliopathy (NPHP-RC) is a common genetic cause of end-stage renal failure during childhood and adolescence and exhibits an autosomal recessive pattern of inheritance. Genetic diagnosis is quite limited owing to genetic heterogeneity in NPHP-RC. We designed a novel approach involving the step-wise screening of Sanger sequencing and targeted exome sequencing for the genet...

2015
Simon Ramsbottom Colin Miles John Sayer Russell J. Ferland Kiet Hua Eugen Boltshauser

The study of primary cilia is of broad interest both in terms of disease pathogenesis and the fundamental biological role of these structures. Murine models of ciliopathies provide valuable tools for the study of these diseases. However, it is important to consider the precise phenotype of murine models and how dependant it is upon genetic background. Here we compare and contrast murine models ...

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