Identification of a novel mutation (C321X) in HJV

Blood. 2004 Oct 1;104(7):2176-7. doi: 10.1182/blood-2004-01-0400. Epub 2004 May 11.

Abstract

Juvenile hemochromatosis is a rare autosomal recessive disorder characterized by the early onset of severe iron overload. We report the occurrence of compound heterozygous mutations in hemojuvelin (HJV), including a termination codon, in a patient with juvenile hemochromatosis but no family history of iron disorders.

Publication types

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

MeSH terms

  • Adult
  • Biopsy
  • Codon, Nonsense
  • DNA Primers / chemistry
  • Family Health
  • Female
  • GPI-Linked Proteins
  • Hemochromatosis / diagnosis*
  • Hemochromatosis / genetics*
  • Hemochromatosis Protein
  • Heterozygote
  • Humans
  • Iron / metabolism
  • Liver / pathology
  • Male
  • Membrane Proteins / genetics*
  • Middle Aged
  • Mutation*
  • Mutation, Missense
  • Protein Structure, Tertiary

Substances

  • Codon, Nonsense
  • DNA Primers
  • GPI-Linked Proteins
  • HJV protein, human
  • Hemochromatosis Protein
  • Membrane Proteins
  • Iron