Patterns of inheritance in familial ALS.
We investigated 185 families with ALS for evidence of anticipation and mitochondrial inheritance. Although initial analysis demonstrated significant anticipation of age at death between generations in patients with familial ALS, further analysis demonstrated features of regression to the mean, suggesting that the perceived differences are the result of bias. In addition, there was no evidence of an effect of preferential maternal inheritance, which would have supported transmission of mitochondrial DNA mutations.
Bradley
Marcus
M
Department of Clinical Neurosciences, Royal Free and University College Medical School, University College London, London, England.
Bradley
Lloyd
L
de Belleroche
Jackie
J
Orrell
Richard W
RW
D017677:Age Distribution; D017668:Age of Onset; D000690:Amyotrophic Lateral Sclerosis / Q000151:congenital / Q000453:epidemiology / Q000235:genetics*; D020132:Anticipation, Genetic / Q000235:genetics*; D015982:Bias; D004252:DNA Mutational Analysis; D004272:DNA, Mitochondrial / Q000235:genetics*; D018450:Disease Progression; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D005820:Genetic Testing; D006801:Humans; D040582:Inheritance Patterns / Q000235:genetics*; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D012737:Sex Factors
D004272:DNA, Mitochondrial