They derive from an adult (19 year old) patient with Frasier Syndrome, which is caused by a mutation in the Wilm’s tumor gene 1 (WT1), resulting in a decrease in WT1 + KTS isoforms due to a disruption of alternative splicing associated with decreased expression of the transcription factors SRY (sex-determining region on Y) and SOX9 (SRY box protein 9) in SCs, affecting their proper maturation [50]. The gene discussed is SOX9; the disease is Frasier syndrome.