Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001387604 | SCV001588275 | pathogenic | T-cell immunodeficiency, congenital alopecia, and nail dystrophy | 2020-07-13 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This sequence change results in a premature translational stop signal in the FOXN1 gene (p.Leu439Argfs*111). While this is not anticipated to result in nonsense mediated decay, it is expected to disrupt the last 210 amino acids of the FOXN1 protein. This variant is not present in population databases (ExAC no frequency). This variant has been observed in individual(s) with autosomal dominant FOXN1 haploinsufficiency (PMID: 31447097). This variant is also known as c.1316_1316delT in the literature. This variant disrupts the C-terminus of the FOXN1 protein. Other variant(s) that disrupt this region (p.Gln489Argfs*61) have been determined to be pathogenic (PMID: 31566583). This suggests that variants that disrupt this region of the protein are likely to be causative of disease. |