Molecular etiology of factor VIII deficiency in hemophilia A

Hum Mutat. 1995;5(1):1-22. doi: 10.1002/humu.1380050102.

Abstract

Hemophilia is a common X-linked coagulation disorder due to deficiency of factor VIII. The factor VIII gene has been cloned in 1984 and a large number of mutations that cause hemophilia A have been identified in the last decade. The most common of the mutations is an inversion of factor VIII that accounts for nearly 45% of patients with severe hemophilia A. This review lists all the factor VIII mutations identified to date and briefly discusses their functional significance.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Amino Acid Sequence
  • Base Sequence
  • Binding Sites / genetics
  • Chromosome Mapping
  • DNA / genetics
  • DNA Mutational Analysis
  • Factor VIII / chemistry
  • Factor VIII / genetics*
  • Factor VIII / metabolism
  • Female
  • Gene Rearrangement
  • Hemophilia A / genetics*
  • Humans
  • Male
  • Mutation
  • Point Mutation
  • Polymorphism, Genetic
  • Retroelements
  • Sequence Deletion
  • X Chromosome / ultrastructure

Substances

  • Retroelements
  • Factor VIII
  • DNA