نتایج جستجو برای: dominant mutation

تعداد نتایج: 404478  

Journal: :The New England journal of medicine 2014
Davide Monteferrario Nikhita A Bolar Anna E Marneth Konnie M Hebeda Saskia M Bergevoet Hans Veenstra Britta A P Laros-van Gorkom Marius A MacKenzie Cyrus Khandanpour Lacramiora Botezatu Erik Fransen Guy Van Camp Anthonie L Duijnhouwer Simone Salemink Brigith Willemsen Gerwin Huls Frank Preijers Waander Van Heerde Joop H Jansen Marlies J E Kempers Bart L Loeys Lut Van Laer Bert A Van der Reijden

The gray platelet syndrome is a hereditary, usually autosomal recessive bleeding disorder caused by a deficiency of alpha granules in platelets. We detected a nonsense mutation in the gene encoding the transcription factor GFI1B (growth factor independent 1B) that causes autosomal dominant gray platelet syndrome. Both gray platelets and megakaryocytes had abnormal marker expression. In addition...

2017
Philip A. Ruzycki Courtney D. Linne Anne K. Hennig Shiming Chen

Purpose The cone-rod homeobox (CRX) transcription factor is essential for photoreceptor gene expression, differentiation, and survival. Human CRX mutations can cause dominant retinopathies of varying onset and phenotype severity. In animal models, dominant frameshift Crx mutations introduce a premature termination codon (PTC), producing inactive truncated proteins that interfere with normal CRX...

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