Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001297765 | SCV001486796 | uncertain significance | Reticular dysgenesis | 2020-03-10 | criteria provided, single submitter | clinical testing | This variant is not present in population databases (ExAC no frequency). This variant has been observed in individual(s) with clinical features of AK2 deficiency (PMID: 31673062). This variant has been reported to affect AK2 protein function (PMID: 31673062). 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. This sequence change replaces serine with proline at codon 208 of the AK2 protein (p.Ser208Pro). The serine residue is weakly conserved and there is a moderate physicochemical difference between serine and proline. |