نتایج جستجو برای: aipl1

تعداد نتایج: 84  

2009
Periasamy Sundaresan P. Vijayalakshmi Stewart Thompson Audrey C. Ko John H. Fingert Edwin M. Stone

PURPOSE To test patients from southern India for the presence of mutations that most commonly cause Leber congenital amaurosis (LCA) in northern America. METHODS A review of the literature identified 177 unique LCA causing mutations in eight different genes: aryl hydrocarbon receptor interacting protein-like 1 (AIPL1), crumbs homolog 1 (CRB1), cone-rod homeobox (CRX), guanylate cyclase 2D (GU...

Journal: :Investigative ophthalmology & visual science 2005
Jana Zernant Maigi Külm Sharola Dharmaraj Anneke I den Hollander Isabelle Perrault Markus N Preising Birgit Lorenz Josseline Kaplan Frans P M Cremers Irene Maumenee Robert K Koenekoop Rando Allikmets

PURPOSE Leber congenital amaurosis (LCA) is an early-onset inherited disorder of childhood blindness characterized by visual impairment noted soon after birth. Variants in at least six genes (AIPL1, CRB1, CRX, GUCY2D, RPE65, and RPGRIP1) have been associated with a diagnosis consistent with LCA or early-onset retinitis pigmentosa (RP). Genetically heterogeneous inheritance complicates the analy...

Journal: :Human mutation 2004
Sylvain Hanein Isabelle Perrault Sylvie Gerber Gaëlle Tanguy Fabienne Barbet Dominique Ducroq Patrick Calvas Hélène Dollfus Christian Hamel Tuija Lopponen Francis Munier Louisa Santos Stavit Shalev Dimitrios Zafeiriou Jean-Louis Dufier Arnold Munnich Jean-Michel Rozet Josseline Kaplan

Leber congenital amaurosis (LCA) is the earliest and most severe form of all inherited retinal dystrophies, responsible for congenital blindness. Disease-associated mutations have been hitherto reported in seven genes. These genes are all expressed preferentially in the photoreceptor cells or the retinal pigment epithelium but they are involved in strikingly different physiologic pathways resul...

Journal: :International Ophthalmology Clinics 2021

Introduction Retinitis pigmentosa (RP) affects between 1 in 3000 and 4000 people 1.77 2.35 million worldwide (https://rarediseases.org/rare-diseases/retinitis-pigmentosa/). The disease is characterized by the death of rod photoreceptor cells leading to defects dark adaptation night blindness. RP typically progresses loss peripheral vision eventually central over a period decades. Half cases occ...

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