Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Johns Hopkins Genomics, |
RCV001260989 | SCV001438365 | uncertain significance | Postaxial polydactyly-anterior pituitary anomalies-facial dysmorphism syndrome | 2020-10-01 | criteria provided, single submitter | clinical testing | This GLI2 variant (rs768928173) is rare (<0.1%) in a large population dataset (gnomAD: 3/233630 total alleles; 0.001%; no homozygotes) and has not been reported previously in the literature to our knowledge. Two bioinformatics tools predict this variant would be tolerated while another predicts it would be damaging. The glutamate at this position is conserved across most species assessed except sarcopterygii, which have aspartate at this position. This variant is not predicted to affect normal exon 13 splicing, although this has not been confirmed experimentally to our knowledge. Due to insufficient evidence, we consider the clinical significance of c.4126G>A to be uncertain at this time. |