L-histidine decarboxylase and Tourette's syndrome

N Engl J Med. 2010 May 20;362(20):1901-8. doi: 10.1056/NEJMoa0907006. Epub 2010 May 5.

Abstract

Tourette's syndrome is a common developmental neuropsychiatric disorder characterized by chronic motor and vocal tics. Despite a strong genetic contribution, inheritance is complex, and risk alleles have proven difficult to identify. Here, we describe an analysis of linkage in a two-generation pedigree leading to the identification of a rare functional mutation in the HDC gene encoding L-histidine decarboxylase, the rate-limiting enzyme in histamine biosynthesis. Our findings, together with previously published data from model systems, point to a role for histaminergic neurotransmission in the mechanism and modulation of Tourette's syndrome and tics.

Publication types

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

MeSH terms

  • Chromosome Mapping
  • Codon, Nonsense*
  • Female
  • Genes, Dominant
  • Genetic Linkage
  • Genetic Predisposition to Disease
  • Haplotypes
  • Histidine Decarboxylase / genetics*
  • Histidine Decarboxylase / metabolism
  • Humans
  • Male
  • Microsatellite Repeats
  • Pedigree
  • Polymerase Chain Reaction
  • Tourette Syndrome / genetics*

Substances

  • Codon, Nonsense
  • Histidine Decarboxylase