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. 1985 Mar;37(2):350–357.

A genetic model for age at onset in Huntington disease.

L A Farrer, P M Conneally
PMCID: PMC1684565  PMID: 3157315

Abstract

Although numerous investigators have confirmed excess paternal transmission among juvenile-onset cases of Huntington disease (HD), there are conflicting reports that the late-onset form is inherited more often from the mother than from the father. Results from a survey of age at onset and age at death in 569 patients corroborate earlier findings of delayed onset of HD among offspring of affected mothers at both ends of the onset-age spectrum: 23 of 28 juvenile-onset offspring had affected fathers, and there were 1.6 times more late-onset offspring born to affected mothers than to affected fathers. These patterns, together with data that link age-at-onset variability to familial longevity trends, suggest a model where age at onset is governed, generally, by a set of independently inherited aging genes, but expression of the HD gene may be significantly delayed in individuals who possess a particular maternally transmitted factor.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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