Protein Kinase, Y-Linked (PRKY)

PRKY encodes a member of the cAMP-dependent serine/threonine protein kinase family. This gene is located on chromosome Y, near the boundary of the pseudoautosomal region. Abnormal recombination between this gene and a related gene on chromosome X is a frequent cause of XX males and XY females.In a collection of 26 XX males and 4 XY females, between 27 and 35% of the interchanges took place between PRK homologs, but at different sites within the gene. the unprecedented high sequence identity and identical orientation of PRKY to its homologous partner on the X chromosome, PRKX, explains the high frequency of abnormal pairing and the subsequent ectopic recombination, leading to XX males and XY females and to the highest rate of recombination outside the pseudoautosomal region.