Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000674085 | SCV000799359 | uncertain significance | Usher syndrome type 1F | 2018-04-16 | criteria provided, single submitter | clinical testing | |
Ce |
RCV001092481 | SCV001249009 | uncertain significance | not provided | 2019-08-01 | criteria provided, single submitter | clinical testing | |
Broad Center for Mendelian Genomics, |
RCV000674085 | SCV003761105 | uncertain significance | Usher syndrome type 1F | 2023-01-24 | criteria provided, single submitter | curation | The p.Pro292Arg variant in PCDH15 has been reported in 1 individual with Usher syndrome type 1F (PMID: 24498627) and has been identified in 0.0009% (1/113578) of European (non-Finnish) chromosomes by the Genome Aggregation Database (gnomAD, http://gnomad.broadinstitute.org; dbSNP ID: rs138744579). Although this variant has been seen in the general population in a heterozygous state, its frequency is low enough to be consistent with a recessive carrier frequency. This variant has also been reported in ClinVar (Variation ID#: 557886) and has been interpreted as a variant of uncertain significance by Counsyl and CeGaT Center for Human Genetics Tuebingen. Computational prediction tools and conservation analyses do not provide strong support for or against an impact to the protein. In summary, the clinical significance of the p.Pro292Arg variant is uncertain. ACMG/AMP Criteria applied: PM2_supporting (Richards 2015). |