نتایج جستجو برای: anophthalmia

تعداد نتایج: 586  

2017
Marina Riera Ana Wert Isabel Nieto Esther Pomares

BACKGROUND Microphthalmia and anophthalmia (MA) are congenital eye abnormalities that show an extremely high clinical and genetic complexity. In this study, we evaluated the implementation of whole exome sequencing (WES) for the genetic analysis of MA patients. This approach was used to investigate three unrelated families in which previous single-gene analyses failed to identify the molecular ...

Journal: :Genesis 2001
P Tucker L Laemle A Munson S Kanekar E R Oliver N Brown H Schlecht M Vetter T Glaser

The eyeless inbred mouse strain ZRDCT has long served as a spontaneous model for human anophthalmia and the evolutionary reduction of eyes that has occurred in some naturally blind mammals. ZRDCT mice have orbits but lack eyes and optic tracts and have hypothalamic abnormalities. Segregation data suggest that a small number of interacting genes are responsible, including at least one major rece...

Journal: :The Journal of Experimental Medicine 1982
C C Leung

A glycoprotein with an apparent 340,000 mol wt (gp 340K) was isolated from rat kidney saline-soluble extract by ammonium sulfate precipitation, DE 52 ion-exchange cellulose chromatography, concanavalin A affinity column, Sephacryl S-300 gel filtration, and discontinuous polyacrylamide gel electrophoresis (PAGE). The relative purity of gp 340K was examined by double immunodiffusion analysis, dis...

2011
Joe Rainger Ellen van Beusekom Jacqueline K. Ramsay Lisa McKie Lihadh Al-Gazali Rosanna Pallotta Anita Saponari Peter Branney Malcolm Fisher Harris Morrison Louise Bicknell Philippe Gautier Paul Perry Kishan Sokhi David Sexton Tanya M. Bardakjian Adele S. Schneider Nursel Elcioglu Ferda Ozkinay Rainer Koenig Andre Mégarbané C. Nur Semerci Ayesha Khan Saemah Zafar Raoul Hennekam Sérgio B. Sousa Lina Ramos Livia Garavelli Andrea Superti Furga Anita Wischmeijer Ian J. Jackson Gabriele Gillessen-Kaesbach Han G. Brunner Dagmar Wieczorek Hans van Bokhoven David R. FitzPatrick

Ophthalmo-acromelic syndrome (OAS), also known as Waardenburg Anophthalmia syndrome, is defined by the combination of eye malformations, most commonly bilateral anophthalmia, with post-axial oligosyndactyly. Homozygosity mapping and subsequent targeted mutation analysis of a locus on 14q24.2 identified homozygous mutations in SMOC1 (SPARC-related modular calcium binding 1) in eight unrelated fa...

Journal: :Journal of neurophysiology 2015
Gaelle S L Coullon Uzay E Emir Ione Fine Kate E Watkins Holly Bridge

Congenital blindness leads to large-scale functional and structural reorganization in the occipital cortex, but relatively little is known about the neurochemical changes underlying this cross-modal plasticity. To investigate the effect of complete and early visual deafferentation on the concentration of metabolites in the pericalcarine cortex, (1)H magnetic resonance spectroscopy was performed...

2017
F. Hernán Moreno-Caviedes Nórida Velez Cuellar Margarita Caicedo Zapata Gabriel Triana Reina Azucena Sánchez

PURPOSE Describe the socio-demographic characteristics of anophthalmic patients examined at specialized centers of four cities in Colombia to know the different causes of eyeball loss. METHOD A transversal retrospective study was done of 511 medical records from the specialized practices of four cities in Colombia. Socio-demographic data of patients who were seen between January 2011 and Dece...

Journal: :Molecular Vision 2009
Nikolas J.S. London Patricia Kessler Bryan Williams Gayle J. Pauer Stephanie A. Hagstrom Elias I. Traboulsi

PURPOSE Microphthalmia, anophthalmia, and coloboma are ocular malformations with a significant genetic component. Rx is a homeobox gene expressed early in the developing retina and is important in retinal cell fate specification as well as stem cell proliferation. We screened a group of 24 patients with microphthalmia, coloboma, and/or anophthalmia for RX mutations. METHODS We used standard P...

Journal: :Veterinary pathology 2000
K Shibuya M Tajima T Nunoya

Bilateral anophthalmia was discovered in a male rat (No. 1) and unilateral anophthalmia was found in the left eye of two female rats (Nos. 2 and 3) derived from a Fischer 344 inbred colony. One male rat (No. 4), a littermate of No. 3, had externally normal eyes, but his left eye had severe retinal atrophy, which was detected by ophthalmoscopy. The eyelids in both eyes of No. 1 and in the left e...

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