Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Broad Center for Mendelian Genomics, |
RCV001724851 | SCV001950227 | likely pathogenic | Retinitis pigmentosa | 2021-04-01 | criteria provided, single submitter | curation | The p.Gln310Ter variant in CDHR1 was identified in an individual with Retinitis pigmentosa, via a collaborative study between the Broad Institute's Center for Mendelian Genomics and the Pierce lab (https://oculargenomics.meei.harvard.edu/labs/pierce-lab/lab-members/). Based on this evidence we have classified this variant as Likely Pathogenic. If you have any questions about the classification please reach out to the Pierce Lab. |
Broad Center for Mendelian Genomics, |
RCV002227535 | SCV002507067 | likely pathogenic | Cone-rod dystrophy 15 | 2022-05-04 | criteria provided, single submitter | curation | The homozygous p.Gln310Ter variant in CDHR1 was identified by our study in 1 individual with cone-rod dystrophy 15. The variant has been reported in 1 individual of unknown ethnicity with cone-rod dystrophy 15 (PMID: 28765526), and was absent from large population studies. This nonsense variant leads to a premature termination codon at position 310, which is predicted to lead to a truncated or absent protein. Loss of function of the CDHR1 gene is a moderately established disease mechanism in autosomal recessive cone-rod dystrophy 15. In summary, although additional studies are required to fully establish its clinical significance, this variant is likely pathogenic. ACMG/AMP Criteria applied: PVS1_strong, PM2, PM3_supporting (Richards 2015). |
OMIM | RCV003222341 | SCV003852637 | pathogenic | Macular dystrophy, retinal, 5 | 2023-03-30 | no assertion criteria provided | literature only |