winged helix deficiency(English)

• failure of the thymus gland to develop results in an immunodeficiency state characterized by a deficiency of T-cells and a secondary humoral immunodeficiency. WHD results from mutations in the winged helix transcription factor, FOXN1, which is expressed, from an early developmental stage, in thymic epithelial cells and in skin structures and is essential for the development of the gland and for normal hair and nail growth. This results in a profound immunodeficiency associated with absence of hair and nail dystrophy aka the nude severe combined immunodeficiency phenotype. See also DiGeorge syndrome
• FOXN1, DGS
• Immunology, Thymus
• https://doi.org…74279-7.18002-6