Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000760444 | SCV000890328 | pathogenic | not provided | 2018-11-01 | criteria provided, single submitter | clinical testing | The W157X variant in the CRYGC gene has been reported previously to segregate with autosomal dominant nuclear cataracts and mircrocornea in a four-generation Chinese family (Zhang et al., 2009). Functional studies with transfected W157X cells demonstrate self-aggregation and reduction in solubility that would be expected to generate light-scattering particles, compromising the transparency of the cells and their assemblies (Talla et. al, 2008). This variant is predicted to cause loss of normal protein function through protein truncation where the last 18 amino acid residues are lost. or nonsense-mediated mRNA decay. The W157X variant is not observed in large population cohorts (Lek et al., 2016). We interpret W157X as a pathogenic variant. |
Miami Human Genetics, |
RCV000056309 | SCV005043114 | pathogenic | Cataract 2, multiple types | 2024-05-09 | criteria provided, single submitter | research | |
OMIM | RCV000056309 | SCV000087478 | pathogenic | Cataract 2, multiple types | 2009-01-01 | no assertion criteria provided | literature only |