نتایج جستجو برای: joubert syndrome

تعداد نتایج: 622071  

2011
Ruxandra Bachmann-Gagescu Ian G. Phelps George Stearns Brian A. Link Susan E. Brockerhoff Cecilia B. Moens Dan Doherty

Ciliopathies are a genetically and phenotypically heterogeneous group of human developmental disorders whose root cause is the absence or dysfunction of primary cilia. Joubert syndrome is characterized by a distinctive hindbrain malformation variably associated with retinal dystrophy and cystic kidney disease. Mutations in CC2D2A are found in ∼10% of patients with Joubert syndrome. Here we desc...

Journal: :Journal of medical genetics 2009
E A Otto K Tory M Attanasio W Zhou M Chaki Y Paruchuri E L Wise M T F Wolf B Utsch C Becker G Nürnberg P Nürnberg A Nayir S Saunier C Antignac F Hildebrandt

BACKGROUND Nephronophthisis (NPHP), a rare recessive cystic kidney disease, is the most frequent genetic cause of chronic renal failure in children and young adults. Mutations in nine genes (NPHP1-9) have been identified. NPHP can be associated with retinal degeneration (Senior-Løken syndrome), brainstem and cerebellar anomalies (Joubert syndrome), or liver fibrosis. METHODS To identify a cau...

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