نتایج جستجو برای: homozygosity mapping

تعداد نتایج: 200921  

2011
Andrew R. Cullinane Thierry Vilboux Kevin O’Brien James A. Curry Dawn M. Maynard Hannah Carlson-Donohoe Carla Ciccone Thomas C. Markello Meral Gunay-Aygun Marjan Huizing William A. Gahl

We evaluated a 32-year-old woman whose oculocutaneous albinism (OCA), bleeding diathesis, neutropenia, and history of recurrent infections prompted consideration of the diagnosis of Hermansky-Pudlak syndrome type 2. This was ruled out because of the presence of platelet δ-granules and absence of AP3B1 mutations. As parental consanguinity suggested an autosomal recessive mode of inheritance, we ...

Journal: :Investigative ophthalmology & visual science 2007
Anneke I den Hollander Irma Lopez Suzanne Yzer Marijke N Zonneveld Irene M Janssen Tim M Strom Jayne Y Hehir-Kwa Joris A Veltman Maarten L Arends Thomas Meitinger Maria A Musarella L Ingeborgh van den Born Gerald A Fishman Irene H Maumenee Klaus Rohrschneider Frans P M Cremers Robert K Koenekoop

PURPOSE Leber congenital amaurosis (LCA) and juvenile retinitis pigmentosa (RP) cause severe visual impairment early in life. Thus far, mutations in 13 genes have been associated with autosomal recessive LCA and juvenile RP. The purpose of this study was to use homozygosity mapping to identify mutations in known LCA and juvenile RP genes. METHODS The genomes of 93 consanguineous and nonconsan...

Journal: :Journal of medical case reports 2015
Siham Chafai-Elalaoui Matthias Chalon Nadia Elkhartoufi Yamna Kriouele Maria Mansouri Tania Attié-Bitach Abdelaziz Sefiani Lekbir Baala

INTRODUCTION Joubert syndrome is a rare congenital disorder characterized by brain malformation, developmental delay with hypotonia, ocular motor apraxia, and breathing abnormalities. Joubert syndrome is a genetically highly heterogeneous ciliopathy disorder with 23 identified causative genes. The diagnosis is based on brain imaging showing the "molar tooth sign" with cerebellar vermis agenesis...

2011
Koichi Hagiwara Hiroyuki Morino Jun Shiihara Tomoaki Tanaka Hitoshi Miyazawa Tomoko Suzuki Masakazu Kohda Yasushi Okazaki Kuniaki Seyama Hideshi Kawakami

Genes involved in disease that are not common are often difficult to identify; a method that pinpoints them from a small number of unrelated patients will be of great help. In order to establish such a method that detects recessive genes identical-by-descent, we modified homozygosity mapping (HM) so that it is constructed on the basis of homozygosity haplotype (HM on HH) analysis. An analysis u...

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