Thirty-seven CAG repeats in the androgen receptor gene in two healthy individuals

J Neurol. 2001 Jan;248(1):23-6. doi: 10.1007/s004150170265.

Abstract

X-linked recessive spinobulbar muscular atrophy (SBMA) is an adult-onset X-linked neurodegenerative disease, characterised by muscular atrophy, bulbar symptoms and endocrinological disturbances. SBMA is caused by the expansion of a CAG repeat in the androgen receptor gene. The maximum number of CAG repeats found in a healthy person is 35 while the minimum number of repeats found in SBMA patients is 38. We have identified a 46-year-old man from an SBMA family with 37 CAG repeats who until now is clinically unaffected. Interestingly, his 85-year-old mother who has the genotype 37/51 CAG repeats is clinically unaffected as well. These results suggest an exactly defined border between normal and disease alleles.

Publication types

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

MeSH terms

  • Aged
  • Female
  • Genotype
  • Humans
  • Male
  • Middle Aged
  • Muscular Atrophy, Spinal / genetics*
  • Muscular Atrophy, Spinal / pathology
  • Pedigree
  • Phenotype
  • Receptors, Androgen / genetics*
  • Trinucleotide Repeat Expansion / genetics*

Substances

  • Receptors, Androgen