Refinement of the chromosome 5p locus for craniometaphyseal dysplasia

Hum Genet. 2001 May;108(5):394-7. doi: 10.1007/s004390100515.

Abstract

Craniometaphyseal dysplasia--Jackson type (CMDJ) is an autosomal dominant bone dysplasia with hyperostosis and sclerosis of the skull and abnormal modelling of the metaphyses. In a large German pedigree, a locus for CMDJ has been mapped previously to the short arm of chromosome 5 (5p15.2-p14.1), defining a 19-cM disease interval between markers D5S2004 and D5S502. Analysis of a large Australian pedigree together with a second German family confirms linkage to the same region. Obligate recombinations in the new families and confirmation of a supposed recombination in the previously reported German kindred have enabled us to narrow the critical region down to approximately 4 cM between markers D5S1987 and D5S1991.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Australia
  • Bone Diseases, Developmental / genetics*
  • Chromosomes, Human, Pair 5 / genetics*
  • Female
  • Genes, Dominant / genetics
  • Genetic Linkage / genetics*
  • Genetic Markers / genetics
  • Germany
  • Haplotypes
  • Humans
  • Lod Score
  • Male
  • Pedigree
  • Recombination, Genetic / genetics
  • Reproducibility of Results

Substances

  • Genetic Markers