Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002036633 | SCV002317676 | uncertain significance | Severe combined immunodeficiency due to DNA-PKcs deficiency | 2021-12-03 | criteria provided, single submitter | clinical testing | This sequence change replaces threonine with isoleucine at codon 3892 of the PRKDC protein (p.Thr3892Ile). The threonine residue is moderately conserved and there is a moderate physicochemical difference between threonine and isoleucine. This variant is present in population databases (rs374822637, gnomAD 0.004%). This variant has not been reported in the literature in individuals affected with PRKDC-related conditions. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |