Screening for inversions in the factor VIII (F8) gene causing severe haemophilia A

Blood Coagul Fibrinolysis. 1994 Apr;5(2):239-42. doi: 10.1097/00001721-199404000-00013.

Abstract

A total of 164 unrelated patients with severe haemophilia A were screened for partial inversions of the factor VIII (F8) gene resulting from recombination between the intronic F8A gene and one or other of two homologous upstream A gene sequences. Inversions were found in 69 (42%) patients. Most inversions (90%) involved the distal rather than the proximal A gene. This unique mutational mechanism is estimated to occur with a frequency of 7.2 x 10(-6) per gene per gamete per generation. Although two patients with an inversion possessed inhibitors (antibodies) against factor VIII, possession of inhibitors did not appear to be associated disproportionately with inversion-type mutations.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Blotting, Southern
  • Chromosome Inversion*
  • Factor VIII / genetics*
  • Female
  • Genetic Testing
  • Hemophilia A / genetics*
  • Humans
  • Male
  • Recombination, Genetic*
  • Reference Values

Substances

  • Factor VIII