نتایج جستجو برای: anophthalmia

تعداد نتایج: 586  

Journal: :Investigative ophthalmology & visual science 2004
Saima Aijaz Brian J Clark Kathleen Williamson Veronica van Heyningen Danny Morrison David Fitzpatrick Richard Collin Nicola Ragge Andrea Christoforou Alison Brown Isabel Hanson

PURPOSE To investigate whether 173 patients with microphthalmia, anophthalmia, and coloboma have mutations in the eye-development gene SIX6. METHODS The two exons of the SIX6 gene were amplified by PCR from patients' genomic DNA and directly sequenced to search for mutations. The PCR products of 75 patients were also analyzed by denaturing high-performance liquid chromatography (DHPLC). RES...

Journal: :Journal of medical genetics 1994
S R Phadke A K Sharma S S Agarwal

A 5 day old male with bilateral anophthalmos, hypospadias, bifid scrotum, micrognathia, and cleft palate with normal chromosomes is described. There have been two case reports with similar clinical manifestations but associated with interstitial deletion of 14q (q22q23). We propose that either our patient represents a new syndrome or has a microdeletion which could not be detected on routine cy...

2011
Robert J. Osborne Jennifer J. Kurinczuk Nicola K. Ragge

PURPOSE Sex determining region Y (SRY)-box 2 (SOX2) anophthalmia syndrome is an autosomal dominant disorder manifesting as severe developmental eye malformations associated with brain, esophageal, genital, and kidney abnormalities. The syndrome is usually caused by de novo mutations or deletions in the transcription factor SOX2. To investigate any potential parental susceptibility factors, we s...

Journal: :Human molecular genetics 2006
Kathleen A Williamson Ann M Hever Joe Rainger R Curtis Rogers Alex Magee Zdenek Fiedler Wee Teik Keng Freddie H Sharkey Niolette McGill Clare J Hill Adele Schneider Mario Messina Peter D Turnpenny Judy A Fantes Veronica van Heyningen David R FitzPatrick

We report heterozygous, loss-of-function SOX2 mutations in three unrelated individuals with Anophthalmia-Esophageal-Genital (AEG) syndrome. One previously reported case [Rogers, R.C. (1988) Unknown cases. Proceedings of the Greenwood Genetic Center. 7, 57.] has a 2.7 Mb deletion encompassing SOX2 and associated with a cryptic translocation t(3;7)(q28;p21.3). The deletion and translocation break...

Journal: :Molecular Vision 2008
Jie Zhou Femida Kherani Tanya M. Bardakjian James Katowitz Nkecha Hughes Lisa A. Schimmenti Adele Schneider Terri L. Young

PURPOSE Mutations in the SOX2 and CHX10 genes have been reported in patients with anophthalmia and/or microphthalmia. In this study, we evaluated 34 anophthalmic/microphthalmic patient DNA samples (two sets of siblings included) for mutations and sequence variants in SOX2 and CHX10. METHODS Conformational sensitive gel electrophoresis (CSGE) was used for the initial SOX2 and CHX10 screening o...

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