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Nonrandom inactivation of the X chromosome in early lineage hematopoietic
cells in carriers of Wiskott-Aldrich syndrome
G Wengler, JB Gorlin, JM Williamson, FS Rosen and DH Bing
Center for Blood Research, Children's Hospital, Boston, MA, USA.
The Wiskott-Aldrich syndrome (WAS) is an X-linked (Xp11.22) recessive
immunodeficiency syndrome characterized by susceptibility to opportunistic
and pyogenic infections, thrombocytopenia, and eczema. Previous studies of
obligate carriers of WAS documented that nonrandom inactivation of the X
chromosome carrying the defective gene is observed in all peripheral blood
cells. The existence of both abnormal platelets and lymphocytes is
consistent with a defect that affects early hematopoietic precursors. We
isolated CD34+ hematopoietic progenitor cells collected from obligate
carriers of WAS by apheresis and used polymerase chain reaction analysis of
a polymorphic variable number of repeats (VNTR) within the X-linked
androgen receptor to document nonrandom inactivation. These data show that
nonrandom inactivation of the X-chromosome in WAS-obligate carriers occurs
early during hematopoietic differentiation.
Volume 85,
Issue 9,
pp. 2471-2477,
05/01/1995
Copyright © 1995 by The American Society of Hematology
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