Investigation of a putative role for FLVCR, a cytoplasmic heme exporter, in Diamond-Blackfan anemia

Blood Cells Mol Dis. 2005 Sep-Oct;35(2):189-92. doi: 10.1016/j.bcmd.2005.01.005.

Abstract

Diamond-Blackfan anemia (DBA) is a rare congenital pure red cell aplasia. Previous studies indicate that mutations of a gene on chromosome 19q13.2, which encodes a ribosomal protein, are responsible for 25% of cases. Recent investigations suggest both the presence of a second candidate region on chromosome 8p and non-19q, non-8p disease. In linkage analysis studies of 28 multiplex DBA families, we identified 8 families with disease linkage to chromosome 1q31. In 4 families, the disease linked exclusively to 1q31. Here, we report that the FLVCR gene on 1q31, which encodes a cytoplasmic heme exporter associated with red cell aplasia in cats, is not involved in DBA in these families.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • 3' Untranslated Regions
  • Anemia, Diamond-Blackfan / etiology
  • Anemia, Diamond-Blackfan / genetics*
  • Chromosomes, Human, Pair 1
  • DNA Mutational Analysis
  • Family Health
  • Genetic Linkage*
  • Heme / metabolism
  • Humans
  • Membrane Transport Proteins / genetics*
  • Membrane Transport Proteins / metabolism
  • Membrane Transport Proteins / physiology
  • Pedigree
  • Point Mutation
  • Receptors, Virus / genetics*
  • Receptors, Virus / metabolism
  • Receptors, Virus / physiology

Substances

  • 3' Untranslated Regions
  • FLVCR1 protein, human
  • Membrane Transport Proteins
  • Receptors, Virus
  • Heme