MATERNAL FACTORS IN ONSET OF HUNTINGTON DISEASE

  • 1 January 1985
    • journal article
    • research article
    • Vol. 37  (3) , 511-523
Abstract
Analyses of father-offspring and mother-offspring similarity in onset age sugget that nuclear genes account for a significant portion of the modification of onset age in Huntington disease. The effects of non-nuclear modifiers are supported by the finding that the offspring of affected women have significantly older mean ages of onset than offspring of affected men irrespective of the onset age in the parent. The absence of increased father-daughter similarity indicates that modification is not X-linked. The absence of reproductive advantage for late-onset individuals and the absence of a multigenerational maternal-lineage effect suggest that the modifying effect of the sex of the affected parent occurs in a single parental generation. Offspring of affected women with onset between ages 35 and 49 had a significantly older mean onset age than their mothers. This suggest that a protective effect may be conferred upon the offspring of affected women.