Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV000933885 | SCV005382674 | likely benign | T-cell immunodeficiency, congenital alopecia, and nail dystrophy | 2024-07-29 | reviewed by expert panel | curation | The NM_001369369.1(FOXN1):c.713G>A (p.Gly238Asp) missense variant has a gnomADv2.1.1 PopMax filtering AF of 0.003236, based on 75/19954 alleles in the East Asian population, which is above the >0.00141 threshold for BS1. Expression vectors containing Foxn1 variants were transfected into HeLa cells along with the β5t-luciferase reporter construct, a well-defined transcriptional target of Foxn1. This variant resulted in 105% activity in the reporter assay compared to WT (PMID: 37419334). After a comprehensive literature search, the variant has not been identified in any individuals with T-cell immunodeficiency, congenital alopecia, and nail dystrophy. In summary, this variant meets criteria to be classified as likely benign for semidominant T-cell immunodeficiency, congenital alopecia, and nail dystrophy based on the ACMG/AMP criteria applied, as specified by the ClinGen SCID VCEP: BS1. |
Labcorp Genetics |
RCV000933885 | SCV001079594 | likely benign | T-cell immunodeficiency, congenital alopecia, and nail dystrophy | 2024-01-25 | criteria provided, single submitter | clinical testing |