Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV000588019 | SCV000696643 | pathogenic | Thrombocytopenia 1 | 2016-12-07 | criteria provided, single submitter | clinical testing | Variant summary: The WAS c.413G>C (p.Arg138Pro) variant located in the WH1/EVH1 domain (via InterPro) causes a missense change involving a non-conserved nucleotide, which 3/5 in silico tools predict a damaging outcome. Multiple functional studies support these predictions. The variant of interest was not observed in controls (ExAC, 1000 Gs, or ESP) and has been reported in multiple affected indivdiuals (1 classic WAS, 2 brothers X-linked thrombocytopenia, 1 mild WAS (indicated to be de novo) via publications, which support the variability of WAS clinical manifestations. One database cites the variant as pathogenic but has not been reported by clinical diagnostic laboratories. Therefore, taking all available lines of evidence into consideration, the variant of interest has been classified as "pathogenic." |