Two models for a maternal factor in the inheritance of Huntington disease.
Huntington disease is a classic example of an autosomal dominant trait. Over the years, however, a number of investigators have reported anomalies regarding the age of onset of the disease that are inconsistent with this paradigm. We propose two models in which a maternal factor--cytoplasmic in one case, autosomal or X-linked in the other--acts to delay onset in a manner consistent with the previously reported anomalies. Relevant data from the Huntington's Disease Research Roster are presented that reinforce and extend the previous observations.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000483:Alleles; D005111:Extrachromosomal Inheritance; D005260:Female; D005799:Genes, Dominant; D008040:Genetic Linkage; D005838:Genotype; D006801:Humans; D006816:Huntington Disease / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D008957:Models, Genetic; D010641:Phenotype; D014960:X Chromosome