Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV003322695 | SCV004027727 | likely pathogenic | Pseudohypoparathyroidism type I A | 2023-07-28 | criteria provided, single submitter | clinical testing | Criteria applied: PP4_MOD,PS4_SUP,PM1_SUP,PM2_SUP,PP2,PP3 |
Prevention |
RCV004723265 | SCV005336103 | uncertain significance | GNAS-related disorder | 2024-05-15 | no assertion criteria provided | clinical testing | The GNAS c.878A>G variant is predicted to result in the amino acid substitution p.Lys293Arg. This variant (also reported as p.K294R in NM_001077488.1) was reported in an individual with pseudohypoparathyroidism 1a (Thiele et al 2015. PubMed ID: 25802881). This variant has not been reported in a large population database (http://gnomad.broadinstitute.org), indicating this variant is rare. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |