Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000613299 | SCV000711760 | pathogenic | Ectodermal dysplasia | 2016-02-19 | criteria provided, single submitter | clinical testing | The c.529+1G>A variant in EDAR has been reported in 2 individuals with hypohydro tic ectodermal dysplasia (Chassaing 2006) and was absent from large population studies. Both individuals were compound heterozygous with a second variant in ED AR. In addition, this variant occurs in the invariant region (+/- 1/2) of the sp lice consensus sequence and is predicted to cause altered splicing leading to an abnormal or absent protein. In summary, this variant meets our criteria to be c lassified as pathogenic for hypohydrotic ectodermal dysplasia in an autosomal re cessive manner. |