Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002530154 | SCV000650762 | likely benign | Dyskeratosis congenita, autosomal dominant 2; Idiopathic Pulmonary Fibrosis | 2023-10-09 | criteria provided, single submitter | clinical testing | |
Ambry Genetics | RCV004559205 | SCV002750498 | uncertain significance | Dyskeratosis congenita | 2022-05-12 | criteria provided, single submitter | clinical testing | The p.R962H variant (also known as c.2885G>A), located in coding exon 12 of the TERT gene, results from a G to A substitution at nucleotide position 2885. The arginine at codon 962 is replaced by histidine, an amino acid with highly similar properties. This amino acid position is conserved. In addition, this alteration is predicted to be tolerated by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
St. |
RCV003230269 | SCV003928111 | uncertain significance | Dyskeratosis congenita, autosomal dominant 2 | 2023-03-15 | criteria provided, single submitter | clinical testing | The TERT c.2885G>A (p.Arg962His) missense change has a maximum subpopulation frequency of 0.0085% in gnomAD v2.1.1 (https://gnomad.broadinstitute.org/). The in silico tool REVEL predicts a benign effect on protein function, but to our knowledge this prediction has not been confirmed by functional studies. To our knowledge, this variant has not been reported in individuals with clinical features of dyskeratosis congenita. In summary, the evidence currently available is insufficient to determine the clinical significance of this variant. It has therefore been classified as of uncertain significance. |