Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV000586443 | SCV000696645 | likely pathogenic | Wiskott-Aldrich syndrome | 2016-07-18 | criteria provided, single submitter | clinical testing | Variant summary: The WAS c.852delC (p.Glu285Argfs) variant results in a premature termination codon, predicted to cause a truncated or absent WAS protein due to nonsense mediated decay (NMD), which are commonly known mechanisms for disease. If the variant escapes NMD, it is expected to truncate GRSGPLPPXP motifs and WH2 domain. Truncations downstream of this position have been classified as pathogenic/likely pathogenic by our laboratory (e.g. c.1271delG, c.1271dupG, c.1456delG, etc.). This variant is absent in approximately 69730 control chromosomes from ExAC. The variant of interest has not, to our knowledge, been reported in affected individuals via publications and/or reputable databases/clinical diagnostic laboratories. Taken together, this variant is currently classified as a Probable Disease Variant (or Likely Pathogenic). |