Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Harry Perkins Institute Of Medical Research, |
RCV004699147 | SCV005200503 | likely pathogenic | Brachydactyly type E1 | 2024-09-02 | criteria provided, single submitter | case-control | The Glu469Lys variant in PTH1R is highly conserved in mammals, absent from gnomAD v4, segregates in a large family, and is predicted to be high impact. Variants in this gene have already been associated with similar phenotypes. Functional studies in HEK-293 cells and mice showed the variant to be moderately LOF. For these reasons, we have classified the variant as likely pathogenic. |