The Danish HD Registry-a nationwide family registry of HD families in Denmark

Clin Genet. 2017 Sep;92(3):338-341. doi: 10.1111/cge.12984. Epub 2017 Mar 28.


The Danish Huntington's Disease Registry (DHR) is a nationwide family registry comprising 14 245 individuals from 445 Huntington's disease (HD) families of which the largest family includes 845 individuals in 8 generations. 1136 DNA and/or blood samples and 18 fibroblast cultures are stored in a local biobank. The birthplace of the oldest HD carrier in each of the 261 families of Danish origin was unevenly distributed across Denmark with a high number of families in the middle part of the peninsula Jutland and in Copenhagen, the capital. The prevalence of HD in Denmark was calculated to be 5-8:100 000. 1451 individuals in the DHR had the size of the HTT CAG repeat determined of which 975 had 36 CAG repeats or more (mean ± SD: 43,5 ± 4,8). Two unrelated individuals were compound heterozygous for alleles ≥36 CAGs, and 60 individuals from 34 independent families carried an intermediate allele.

Keywords: Denmark; HTT CAG repeat; Huntington's disease; family registry.

MeSH terms

  • Age Factors
  • Alleles
  • Biological Specimen Banks
  • Denmark / epidemiology
  • Family
  • Female
  • Geography, Medical
  • Humans
  • Huntingtin Protein / genetics
  • Huntington Disease / diagnosis
  • Huntington Disease / epidemiology*
  • Huntington Disease / genetics
  • Male
  • Registries
  • Trinucleotide Repeat Expansion
  • Trinucleotide Repeats


  • Huntingtin Protein