UNLABELLED Bestrophin, an integral membrane protein existing in basolateral region of the retina is a propitious target for drug discovery. Mutations in the Bestrophin protein cause Best Vitelliform Macular Dystrophy (BVMD) leading to retinal damages and loss of visual acuity. Owing to the lack of three dimensional structure and related structural homologs in the protein data bank, we modeled t...

OBJECTIVE To describe the clinical and genetic findings in 15 patients with multifocal vitelliform lesions. METHODS All patients and, if possible, affected family members underwent an ophthalmic examination and their genomic DNA was analyzed for mutations in the vitelliform macular dystrophy 2 (VMD2) gene. Patients who did not have a mutation in the VMD2 gene were screened for mutations in th...

BACKGROUND Adult-onset foveomacular vitelliform dystrophy is characterized by a solitary, oval, slightly elevated, yellowish subretinal lesion of the fovea. We examined a patient with adult-onset foveomacular vitelliform dystrophy with stellate retinal folds by optical coherence tomography and scanning laser ophthalomoscopy. CASE A 58-year-old Japanese woman with a complaint of decreased visi...

CASE REPORT A 70-year-old male patient diagnosed with bilateral adult-onset vitelliform dystrophy presented with a sudden decrease of vision in his left eye associated with the appearance of an occult type of neovascular membrane. It was treated with intravitreal ranibizumab due to juxtafoveal location of the membrane. Two injections were needed to induce total regression of the lesion. DISCU...

PURPOSE Adult-onset vitelliform macular dystrophy (AVMD) is a pleomorphic late-onset macular phenotype characterized by a central yellow deposit between the neural retina and retinal pigment epithelium. Mutations in the genes encoding peripherin/RDS and VMD2 have been previously reported in some subjects with AVMD. The purpose of this investigation was to determine the prevalence of mutations i...

Security technology is important to security, but the practices of the people who develop, integrate, evaluate, configure, maintain, and use that technology are more important; indeed, these practices are the foundation of technical (as well as physical and personnel) security. It is crucially important, therefore, that security practices be good ones; when feasible, best security practices (BS...

PURPOSE To investigate the multimodal morphological features in the different stages of Best vitelliform macular dystrophy (VMD) in subjects harboring mutations in the BEST1 gene, and their changes during the progression of the disease. METHODS In this retrospective observational study performed between January 2007 and December 2012, 21 patients (42 eyes) with Best VMD from eight families wi...

PURPOSE To characterize outer retina structure in best vitelliform macular dystrophy (BVMD) and to determine the effect of macular lesions on overlying and adjacent photoreceptors. METHODS Five individuals with BVMD were followed prospectively with spectral domain optical coherence tomography and confocal and nonconfocal split-detector adaptive optics scanning light ophthalmoscopy (AOSLO). Th...