نتایج جستجو برای: lebers congenital amaurosis

تعداد نتایج: 120868  

Journal: :The New England journal of medicine 2008
Albert M Maguire Francesca Simonelli Eric A Pierce Edward N Pugh Federico Mingozzi Jeannette Bennicelli Sandro Banfi Kathleen A Marshall Francesco Testa Enrico M Surace Settimio Rossi Arkady Lyubarsky Valder R Arruda Barbara Konkle Edwin Stone Junwei Sun Jonathan Jacobs Lou Dell'Osso Richard Hertle Jian-xing Ma T Michael Redmond Xiaosong Zhu Bernd Hauck Olga Zelenaia Kenneth S Shindler Maureen G Maguire J Fraser Wright Nicholas J Volpe Jennifer Wellman McDonnell Alberto Auricchio Katherine A High Jean Bennett

Leber's congenital amaurosis (LCA) is a group of inherited blinding diseases with onset during childhood. One form of the disease, LCA2, is caused by mutations in the retinal pigment epithelium-specific 65-kDa protein gene (RPE65). We investigated the safety of subretinal delivery of a recombinant adeno-associated virus (AAV) carrying RPE65 complementary DNA (cDNA) (ClinicalTrials.gov number, N...

2014
Anna M. Siemiatkowska L. Ingeborgh van den Born Maria M. van Genderen Mette Bertelsen Ditta Zobor Klaus Rohrschneider Ramon A.C. van Huet Siska Nurohmah B. Jeroen Klevering Susanne Kohl Sultana M.H. Faradz Thomas Rosenberg Anneke I. den Hollander Rob W.J. Collin Frans P.M. Cremers

PURPOSE The gene encoding nicotinamide nucleotide adenylyltransferase 1 (NMNAT1) was recently found to be mutated in a subset of patients with Leber congenital amaurosis (LCA) with macular atrophy. The aim of this study was to determine the occurrence and frequency of NMNAT1 mutations and associated phenotypes in different types of inherited retinal dystrophies. METHODS DNA samples of 161 pat...

Journal: :The Journal of clinical investigation 2011
Manzar Ashtari Laura L Cyckowski Justin F Monroe Kathleen A Marshall Daniel C Chung Alberto Auricchio Francesca Simonelli Bart P Leroy Albert M Maguire Kenneth S Shindler Jean Bennett

Leber congenital amaurosis (LCA) is a rare degenerative eye disease, linked to mutations in at least 14 genes. A recent gene therapy trial in patients with LCA2, who have mutations in RPE65, demonstrated that subretinal injection of an adeno-associated virus (AAV) carrying the normal cDNA of that gene (AAV2-hRPE65v2) could markedly improve vision. However, it remains unclear how the visual cort...

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