Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV001787431 | SCV002029250 | likely pathogenic | Usher syndrome type 2A | 2021-12-06 | criteria provided, single submitter | clinical testing | The variant c.199T>G (p.(Cys67Gly)) in exon 2 of the USH2A-gene is not found in the gnomAD database, it affects a moderately conserved nucleotide, a moderately conserved amino acid within a protein domain and there is a large physicochemical difference between Cys and Gly. This variant has a pathogenic computational verdict based on 8 pathogenic predictions from BayesDel_addAF, EIGEN, FATHMM-MKL, M-CAP, MVP, MutationAssessor, MutationTaster and SIFT vs 4 benign predictions from DANN, DEOGEN2, LIST-S2 and PrimateAI. This variant was found in trans with a known pathogenic variant (c.2299del, p.(Glu767Serfs*21)) in our patient suspected to have an Usher syndrome. ACMG criteria used for classification: PM2, PM3, PP3, PP2. |