Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
New York Genome Center | RCV001591703 | SCV001815741 | uncertain significance | Coffin-Siris syndrome 7 | 2020-11-17 | criteria provided, single submitter | clinical testing | The c.900C>T (p.Arg300=) variant identified in the DPF2 gene is a synonymous variant at amino acid 300/392. The Cytosine at this position is well conserved throughout mammals. While Splice AI does not predict an alteration to splicing, the Transcript inferred Pathogenicity Score (TraP) for this position is 0.381, which is >95% score-percentile for coding variants suggesting it is possibly damaging. The c.900C>T (p.Arg300=) variant is found with low frequency in gnomAD(v3.1) (2 heterozygotes, 0 homozygotes; allele frequency: 1.3e-5), suggesting it is not a common benign variant in the populations represented in that database. This variant is absent from ClinVar and to our current knowledge has not been reported in affected individuals in the literature. Given the lack of compelling evidence for its pathogenicity, the c.900C>T (p.Arg300=) variant identified in the DPF2 gene is reported as a Variant of Uncertain Significance. |