Blood Journal
Leading the way in experimental and clinical research in hematology

Kx: its relationship to chronic granulomatous disease and genetic linkage with Xg

  1. P Densen,
  2. S Wilkinson-Kroovand,
  3. GL Mandell,
  4. G Sullivan,
  5. R Oyen, and
  6. WL Marsh

Abstract

The relationship between neutrophil function and the neutrophil antigen, Kx, as well as the linkage of the gene, Xk, with Xg was examined in a kindred with X-linked chronic granulomatous disease. Four of the eight male siblings had chronic granulomatous disease by clinical history and tests of neutrophil function, and all four had Kx- negative neutrophils. The remaining four were in good health and had normal nitroblue tetrazolium reduction tests. However, one of these latter four had Kx-negative neutrophils that functioned normally. These data suggest that closely linked but distinct genes on the X chromosome code for chronic granulomatous disease and Kx. In addition, close linkage was demonstrated between Xk and Xg, a gene coding for an erythrocyte surface antigen.