Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001522504 | SCV005382650 | likely benign | T-cell immunodeficiency, congenital alopecia, and nail dystrophy | 2024-07-29 | reviewed by expert panel | curation | The NM_001369369.1(FOXN1):c.1664C>T (p.Ala555Val) missense variant has a gnomADv2.1.1 PopMax filtering AF of 0.003187 based on 77/19952 alleles in the East Asian population, which is above the >0.00141 threshold for BS1. No deleterious effect is predicted with a REVEL score of 0.197 meeting the threshold of <0.290 for BP4. Additionally, no splicing effect is predicted by SpliceAI (delta score 0.00). 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, BP4. |
Labcorp Genetics |
RCV001522504 | SCV001732065 | benign | T-cell immunodeficiency, congenital alopecia, and nail dystrophy | 2024-01-31 | criteria provided, single submitter | clinical testing |