How One Woman Brought the 'Mother's Curse' to Canada

[D J Thornton]

How One Woman Brought the 'Mother's Curse' to Canada


Shttps://www.theatlantic.com/science/ar ... da/540153/

o well-documented is French Canadian genealogy that professional geneticists and demographers use the data for research, too. Whenever a small group of people leave a large population (France) to found a new one (New France), they bring with them a particular set of mutations. Some of these mutations will by chance be more common in the new population and others less so. As a result, some rare genetic disorders disproportionately impact French Canadians.
One of these is Leber’s hereditary optic neuropathy, which causes vision loss, usually in young men. Recently, geneticists using French Canadian genealogy have reexamined the effects of Leber’s and found a striking pattern of inheritance: It seems to show a long-theorized but never-seen-in-humans pattern called the “mother’s curse.

[D J Thornton]

The Genetic History of French Canada – ...
https://abbypaige.com › genetics
Mar 5, 2013 · There are higher rates in Quebec of some 30 diseases with a genetic basis, including cystic fibrosis, Tay-Sachs, and certain types of muscular dystrophy, high cholesterol, and rickets. Doctors do, in fact, recommend screening for some of these conditions for those with French-Canadian ancestry.

[D J Thornton]

Human genetics: lessons from Quebec populations.

Scriver CR1.
Author information
Abstract
The population of Quebec, Canada (7.3 million) contains approximately 6 million French Canadians; they are the descendants of approximately 8500 permanent French settlers who colonized Nouvelle France between 1608 and 1759. Their well-documented settlements, internal migrations, and natural increase over four centuries in relative isolation (geographic, linguistic, etc.) contain important evidence of social transmission of demographic behavior that contributed to effective family size and population structure. This history is reflected in at least 22 Mendelian diseases, occurring at unusually high prevalence in its subpopulations. Immigration of non-French persons during the past 250 years has given the Quebec population further inhomogeneity, which is apparent in allelic diversity at various loci. The histories of Quebec's subpopulations are, to a great extent, the histories of their alleles. Rare pathogenic alleles with high penetrance and associated haplotypes at 10 loci (CFTR, FAH, HBB, HEXA, LDLR, LPL, PAH, PABP2, PDDR, and SACS) are expressed in probands with cystic fibrosis, tyrosinemia, beta-thalassemia, Tay-Sachs, familial hypercholesterolemia, hyperchylomicronemia, PKU, oculopharyngeal muscular dystrophy, pseudo vitamin D deficiency rickets, and spastic ataxia of Charlevoix-Saguenay, respectively) reveal the interpopulation and intrapopulation genetic diversity of Quebec. Inbreeding does not explain the clustering and prevalence of these genetic diseases; genealogical reconstructions buttressed by molecular evidence point to founder effects and genetic drift in multiple instances. Genealogical estimates of historical meioses and analysis of linkage disequilibrium show that sectors of this young population are suitable for linkage disequilibrium mapping of rare alleles. How the population benefits from what is being learned about its structure and how its uniqueness could facilitate construction of a genomic map of linkage disequilibrium are discussed.
https://www.ncbi.nlm.nih.gov/pubmed/11701644

[D J Thornton]

Very technical paper regarding heart disease and French Canadian study

Genetic investination of vascular diseases in the French ... - Bibliothèque et Archives Canada
www.collectionscanada.gc.ca › obj › tape2
by C Fournier
Genetic investination of vascular diseases in the French-Canadian ~ o ~ u l a t i o o. Caroline Fournier. Department o f Biology. McGill University, Montreal.