Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genetic Services Laboratory, |
RCV001819306 | SCV002069430 | likely pathogenic | not provided | 2018-12-03 | criteria provided, single submitter | clinical testing | DNA sequence analysis of the GNAS gene demonstrated a sequence change, c.823A>G, in exon 10 that results in an amino acid change, p.Ser275Gly. This sequence change does not appear to have been previously described in patients with GNAS-related disorders and has also not been described as a known benign sequence change in the GNAS gene. The p.Ser275Gly change affects a moderately conserved amino acid residue located in a domain of the GNAS protein that is known to be functional. In-silico pathogenicity prediction tools (SIFT, PolyPhen2, Align GVGD, REVEL) provide contradictory results for the p.Ser275Gly substitution. The c.823A>G sequence change appears to be a de novo event in one patient. |