Origin and diffusion of the myotonic dystrophy gene in the Saguenay region (Quebec). Bouchard, G., Roy, R., Declos, M., Mathieu, J., & Kouladjian, K. Can J Neurol Sci, 16(1):119-122, 1989.
Origin and diffusion of the myotonic dystrophy gene in the Saguenay region (Quebec) [link]Website  abstract   bibtex   
A very high prevalence (approximately 1/475 in 1985) of myotonic dystrophy (Steinert disease) is observed in the Saguenay region, which is located in the north-east part of the Province of Quebec. For various reasons, however, the literature on the subject generally associates a high degree of selective disadvantage with this gene, which seems to contradict the Saguenay data. Using a computerized regional population register, we have reconstituted patients' genealogies and family biographies. We have thus been able to study the origin of the gene and to compare the demographic behavior of patients and controls. On the whole, patients seem to be very little disadvantaged compared to controls, in terms of reproduction as well as of geographical and occupational mobility.
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 title = {Origin and diffusion of the myotonic dystrophy gene in the Saguenay region (Quebec)},
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 year = {1989},
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 keywords = {Female,Humans,Male,Myotonic Dystrophy/epidemiology/*genetics,Non-U.S. Gov't,Quebec,Research Support},
 pages = {119-122},
 volume = {16},
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 abstract = {A very high prevalence (approximately 1/475 in 1985) of myotonic dystrophy (Steinert disease) is observed in the Saguenay region, which is located in the north-east part of the Province of Quebec. For various reasons, however, the literature on the subject generally associates a high degree of selective disadvantage with this gene, which seems to contradict the Saguenay data. Using a computerized regional population register, we have reconstituted patients' genealogies and family biographies. We have thus been able to study the origin of the gene and to compare the demographic behavior of patients and controls. On the whole, patients seem to be very little disadvantaged compared to controls, in terms of reproduction as well as of geographical and occupational mobility.},
 bibtype = {article},
 author = {Bouchard, Gérard and Roy, R and Declos, M and Mathieu, J and Kouladjian, K},
 journal = {Can J Neurol Sci},
 number = {1}
}

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