LOCALIZATION OF THE GENE FOR WIEACKER-WOLFF-SYNDROME IN THE PERICENTROMERIC REGION OF THE X-CHROMOSOME

The Wieacker-Wolff syndrome (WWS, MIM 314580), first described clinic ally in 1985, is an X-linked recessive disorder. In earlier studies, l inkage between the WWS gene and DXYS1 at Xq21.2, and DXS1 at Xq11 as w ell as AR at Xq12 was reported. Here we report on a linkage analysis u sing highly polymorphic, short terminal repeat markers located in the segment from Xp21 to Xq24. No recombination between the WWS locus and ALAS2 or with AR (z = 4.890 at theta = 0.0) was found. Therefore, the WWS locus was assigned to a segment of approximately 8 cM between PFC (Xp11.3-Xp 11.23) and DXS339 (Xq11.2-Xq13).