Gene · 2018 · 11 citations · 15 references
MedicineGeneticsMolecular GeneticsHuman GeneticsOcular DiseaseOcular GeneticsCongenital CataractCongenital Eye AnomaliesEye DevelopmentLens DiseaseAutosomal Dominant Congenital CataractCrystallin Gene MutationsExome SequencingHydrophobicityMissense MutationsMutationMutation SpectrumProtein Translocation
Congenital cataract is a clinically and genetically heterogeneous disease. In this study, we examined a five-generation Chinese family with autosomal dominant nuclear congenital cataracts by whole exome sequencing. A novel heterozygous missense mutation c.199T>A, p.(Tyr67Asn) in CRYGS was identified in this family. The p.(Tyr67Asn) substitution was predicted to decrease the local hydrophobicity and affect the three-dimensional structure of γS-crystallin, and resulted in a portion of mutant protein translocation from the cytoplasm to cell membrane. Our observations expand the mutation spectrum of CRYGS and provide further evidence for the genetic basis and molecular mechanism of congenital cataract.
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Childhood blindness in the context of VISION 2020--the right to sight.
Clare Gilbert, Allen Foster · PubMed · 2001 · 1.1K citations · Full text
Medicine, Ophthalmology, Pediatrics +17
Crystallin gene mutations in Indian families with inherited pediatric cataract.
Ramachandran Ramya Devi, Wenliang Yao, P Vijayalakshmi et al. · PubMed · 2008 · 158 citations
The tyrosine corner: A feature of most greek key β‐barrel proteins
Jens Hemmingsen, Kim M. Gernert, Jane S. Richardson et al. · Protein Science · 1994 · 131 citations · Full text