Hereditary disorders in the French Canadian population of Quebec. II. Contribution of Perche

De Braekeleer, Marc

In our previous paper (De Braekeleer and Dao 1994; this issue of Human Biology), we showed that 24 of 28 hereditary disorders cluster in eastern Quebec, most of them in the northeastern part of the province. Most of these disorders were brought to Nouvelle-France by settlers coming from France in the seventeenth century. We also showed that only 249 of the 8500 settlers in Nouvelle-France contributed, as common founders, to these genealogies. The contribution of the French province of Perche was statistically significantly higher than expected from its relative weight in the founding nucleus of the French Canadian population in 19 disorders. Furthermore, 34 common founders from Perche were found in more than 1 disorder, and 7 were found in 10 disorders or more (De Braekeleer and Dao 1994).

In this article we try to understand why the province of Perche, which contributed only 217 of the 8500 settlers of Nouvelle-France, played such a major role in the development of the French Canadian population. To achieve this objective, we reviewed the historical, social, genealogical, and demographic data. As a first step, several characteristics of the settlers from Perche were compared with those of the settlers from three other French provinces. Second, the characteristics of the settlers from Perche who were common founders in the hereditary disorders were compared with those of the settlers who were not common founders in these disorders.

MATERIALS AND METHODS

To understand the involvement of the founders originating from Perche, we compared them to founders from the Maine, Normandie, and Poitou provinces of France. Maine was chosen because some founders from that province were found more often than expected in several disorders (De Braekeleer and Dao 1994). Poitou and Normandie were chosen because, besides Ile de France (including Paris) (1094 settlers), they provided the largest number of individuals to the founding nucleus of the French Canadian population (750 and 1111, respectively). Ile de France was not chosen because in the seventeenth and eighteenth centuries, with the advent of Louis XIV and his successors, Paris attracted large numbers of individuals coming from all parts of France and from outside France. Furthermore, Ile de France did not contribute a significantly higher number of common founders to the hereditary disorders reported by De Braekeleer and Dao (1994).

Here, a settler is defined as an individual who migrated permanently to North America and participated in the setting up of the French colony. Charbonneau et al. (1987) estimate that, from the origins of the colony to 1730, 340 of the 3380 so-called settlers did not have a child born in Nouvelle-France. A founder is defined as an individual who settled in Nouvelle-France, who had at least one child who got married (usually), and who had at least one liveborn grandchild. This restriction was used to ensure that any individual considered a founder had a chance to contribute to the genetic pool of the French Canadian population.

Data on the place of origin of each founder were collected, as were the type of migration (kin or isolated), the size of the families migrating to Nouvelle-France, the year of marriage of the founders, the place of origin in France of the spouses when they were also founders, the place of settlement in Nouvelle-France, and their total descendance up to December 31, 1729. The last piece of information was taken from Charbonneau et al. (1987). The year of marriage was preferred to the date of arrival because the date of arrival could not always be found in the sources available to us. The place of settlement was defined as the location where most of the children of a given founder were born.

Demographic and genealogical data were extracted from numerous sources. They included dictionaries (Tanguay 1975; Charbonneau and Legar 1980; Jette 1983; Institut Genealogique Drouin 1979; Brown et al. 1967; Seguin 1972), marriage repositories [e.g., Talbot (1948, 1977)], and books and theses analyzing the origins of the French Canadian population (Charbonneau et al. 1987; Boleda 1983; Charbonneau 1975; Henripin 1954; Lanctat 1952; Dumas 1972; Landry 1992; Trudel 1983). Other sources dealt with the history of Quebec, mainly from the origins of the French colony to 1759, the year of the takeover by the English, and with the social aspects of this population (Dickinson and Young 1992; Delge 1991; Harris and Matthews 1987; Brown 1987; Trudel 1966, 1974, 1979; Mathieu 1991; Depatie et al. 1987; Gauvreau 1986). Numerous papers were also consulted [e.g., Debien (1952a,b), Leclerc (1959a-c, 1960a,b), Trudel (1969), Jette (1972), Courville (1984), Beauregard et al. (1986), and Molloy (1990)], as were books written on Perche and the founders originating from that province (Charbonneau 1970; Montagne 1965). Periodicals were also screened for interesting data, notably, the Revue d’Histoire de l’Amerique Francaise, the Rapport de l’Archiviste de la Province de Qeubec, and the Memoires de la Societe Genealogique Canadienne-Francaise, and other French Canadian and Acadian genealogical research was considered.

Only the founders who immigrated to Nouvelle-France before 1730 were considered here because data on the place of origin and the descendance of the founders were sometimes available only until 1730 (Jette 1983; Charbonneau et al. 1987). Furthermore, the data reported in the other sources usually ended in 1760 (Charbonneau and LCgar 1980; Institut Genealogique Drouin 1979; Tanguay 1975). Therefore, because of our restricted definition of a founder, we had to allow at least a 30-year period before the information on the descendants ran out. Such a limitation could have introduced a bias in the analysis. However, only 5 of the 217 individuals from Perche who settled in Nouvelle-France arrived after 1730. Therefore, using 1730 as the cutoff point had only a minor impact on the results.

Because only 48 of the 217 settlers coming from Perche were found in the hereditary disorders studied earlier (De Braekeleer and Dao 1994), we wanted to know whether differences existed between those who contributed to the founding nucleus of the hereditary disorders and those who did not. Therefore the pedigrees of most of the settlers coming from Perche were reconstructed on at least four generations. The spouses and their places of origin were identified; the places of settlement of their descendants were searched to look for possible differences between both groups.

RESULTS AND DISCUSSION

Differential Contribution of Perche to the Genetic Pool.

Complete information was retrieved from the various sources on 72.8% of the settlers from Perche, 56.9% of the settlers from Maine, 66.9% of the settlers from Poitou, and 57.1% of the settlers from Normandie. These differences are the result of the restricted definition of a founder (an individual having had at least one liveborn grandchild). Furthermore, we considered only the founders who settled in Nouvelle-France before 1730. Table 1 shows that the contribution of the four French provinces was not randomly distributed over time (Charbonneau and Robert 1987). (Table 1 omitted.)

Figure 1 shows the distribution of the places of origin of the founders from each province. (Figure 1 omitted.) The places of origin were grouped into the different territorial subdivisions to make the maps easier to read. The places of origin were scattered in the whole territory of Maine, Poitou, and Normandie. On the contrary, the places of origin of the founders from Perche clustered in the territorial subdivision of Mortagne (149/158; 94.3%). Moreover, as shown in Figure 2, most of the founders came from a few small towns and villages: Tourouvre, Mortagne, Belleme, and Ige. The distance between Tourouvre and Ige is only 21.8 mi. (Figure 2 omitted.) It should also be noted that St. Cosme-en-Vairais, located in the province of Maine, is only 4.2 mi away from Ige. Charbonneau (1970) reported that Tourouvre was the rural parish that contributed the most individuals to Nouvelle-France.

The size of the founding families was also statistically different between the four provinces of interest (analysis of variance: F = 50.36, p = 0.0001) (Table 1). Again, in calculating the size of the families, we counted only those founders who had at least a liveborn grandchild. It is agreed that immigrants to Nouvelle-France were mostly isolated individuals. Only 13% of the permanent settlers had relations beyond the nuclear family among other settlers (Guillemette and Legar 1989). Couples married in France accounted for only 250 families (Charbonneau and Robert 1987; Charbonneau et al. 1987). Our results show that major differences exist between provinces (Table 1). The degree of relationship is similar in all four groups; the family groups mostly involve sibs or parents with children, or more rarely uncles and aunts and their nephews and nieces or cousins.

During the French regime, Nouvelle-France was administratively divided into three governments: Quebec, Trois-Rivieres, and Montreal. The Quebec government was the oldest and the Trois-Riveires government the smallest. The place of settlement of the founders was analyzed according to the territory administrated by these three governments. A nonrandom distribution was found (X sup 2 = 35.5, d.f.= 6, p

Thirty-seven of the 158 founders (23.4%) from Perche settled on the Cote de Beaupre (Figure 3). This region, located east of Quebec City, on the north shore of the St. Lawrence River, contributed 151 of the 599 (25.2%) settlers of the Charlevoix region (Jette et al. 1991), which in turn contributed almost 80% of the 11,000 immigrants to Saguenay-Lac-St-Jean between 1838, the date of its opening to white settlement, and 1870 (Gauvreau and Bourque 1991; Gauvreau et al. 1991; De Braekeleer 1991a). At present, most of the hereditary disorders that have a high frequency in the French Canadian population have been shown to cluster in northeastern Quebec (Saguenay-Lac-St.-Jean, Charlevoix, and Cote Nord) (De Braekeleer 1991a; De Braekeleer and Dao 1994). Of these 37 founders, who, as a matter of fact, all came from Mortagne, 26 have been identified as common founders in more than one disorder (De Braekeleer and Dao 1994).

Several founders were married in Nouvelle-France to other founders. Ten of the 88 (11.4%) founders from Perche who got married in Nouvelle-France to another founder chose someone from Perche. Two of the 44 (4.5%) founders from Maine got married to an immigrant also coming from Maine. Marriages in Nouvelle-France between founders who both came from Normandie represented 28.7% (80/279) of all the marriages, and marriages involving two founders from Poitou accounted for 13.1% (25/191) of all marriages. Although statistically significant differences were found in the distribution of the provinces of origin of the immigrants who married founders from the four provinces of interest (p

A significant difference in the mean year of marriage of the founders was found among the four provinces of origin (analysis of variance:

F = 58.12, p = 0.0001) (Table 1). The Scheff procedure, which is a multiple comparison test, showed that most of the significance level is the result of significant differences (p

A significant difference in the mean number of descendants until December 31, 1729, was found among the four provinces (analysis of variance: F = 36.55, p = 0.0001) (Table 1). Again, the Scheff procedure showed that the small overall p value is mainly due to significant differences in the mean number of descendants between Perche and each of the other three provinces. However, it should be noted that the number of descendants on December 31, 1729, was calculated only for the founders who arrived in Nouvelle-France before 1680 (Charbonneau et al. 1987).

Charbonneau et al. (1987) reported the total number of descendants until December 31, 1729, of all 1955 male and 1425 female settlers established in Nouvelle-France before 1680. Of the 50 male settlers who had the greatest number of descendants, 14 came from Perche, 1 came from Maine, 10 came from Normandie, and 1 came from Poitou. Among the 50 female settlers with the greatest descendance, 9 were from Perche, 1 was from Maine, 7 were from Normandie, and 2 were from Poitou. Fifteen of the 34 founders from Perche found to be common in more than one hereditary disorder (De Braekeleer and Dao 1994) were included in the 100 settlers with the greatest number of descendants; none of the 4 founders from Maine, 6 of the 21 founders from Normandie, and 1 of the 10 founders from Poitou were common in more than one hereditary disorder.

The measure of the number of descendants consisted in calculating over several generations the number of individuals issued from one founder. However, one has to keep in mind that any given descendant was usually related to several founders and therefore was counted several times. Charbonneau et al. (1987) calculated that each settler who arrived in Nouvelle-France before 1860 had an average of 88 descendants by the end of 1729, representing some 300,000 individuals. The exact number of descendants issued from all the settlers established before 1680 was 53,234 on December 31, 1729 (which represents a mean of 15.8 descendants per settler).

Another approach consists in calculating the genetic contribution of each founder. The genetic contribution of the founders from Perche who arrived in Nouvelle-France before 1680 accounted for 7.6% of the total genetic contribution of all the founders, although they represented only 4.7% of the founding contingent. The genetic contribution of the founders from Normandie, Ile de France, and the other provinces of midwestern France (Poitou, Aunis, Saintonge, Bretagne, etc.) was consistent with their relative contribution to the founding nucleus established before 1680 (19.1% vs. 18.3% for Normandie, 17.5% vs. 18.0% for Ile de France, and 28.0% vs. 28. 1% for the other midwestern provinces). However, the genetic contribution of the other provinces was less than expected from their relative weight in the founding set before 1680 (19.5% vs. 22.7%) (Charbonneau et al. 1987).

Fifty-eight percent (4958/8527) of the total immigration to Nouvelle-France took place between 1680 and 1759. However, there were only 228 women (4.6%) among them (Charbonneau and Robert 1987). Therefore the male newcomers had to marry female descendants of the settlers who arrived before 1680, thereby increasing their relative genetic contribution. It was estimated that in 1950 the 3274 settlers established before 1680 still accounted for 68% of the total genetic contribution (Charbonneau 1984). Among them, 575 contributed one-third of the genetic pool of the French Canadian population. Furthermore, 70 couples had such a large number of descendants in 1730 that they still account for 15% of the genetic pool of this population (Charbonneau et al. 1987). Eleven of these 70 couples had more than 1000 descendants in 1729. Nine of the 22 founders involved in these 11 couples were from Perche; they included two couples married in 1615 and 1616 with more than 2000 descendants in 1729. Of the 140 spouses (70 couples), 33 were from Perche, 3 were from Maine, 20 were from Normandie, and 5 were from Poitou.

In conclusion, the founders from Perche got married earlier, had a larger number of descendants, and settled in greater number in eastern Quebec than those of the three other provinces studied. Most of the founders came from a limited region of Perche called Mortagne, and they tended to arrive in Nouvelle-France in large familial groups. Finally, the genetic contribution of the founders from Perche established before 1680 was higher than expected from their relative weight in the founding nucleus of the French Canadian population and presumably remained high even in the present-day population. These results can be explained by historical, social, and demographic data.

Dynamics and Process of Colonization in Nouvelle-France.

Colonization of the St. Lawrence River valley started at the beginning of the seventeenth century. The Companie des Cent-Associes, which received the mandate to develop the land, divided the land into lots known as seigneuries (Harris 1966; Dechene 1987). These lots were usually distributed to individuals of noble birth, merchants, and religious figures, rarely to ordinary people. In return, the landowners were asked to develop the land by attracting settlers. These landlords, known as seigneurs, engaged farmers and artisans (so-called commoners hereafter) in France. This seigneurial system had an important influence on the social life in Nouvelle-France. The seigneurs had privileges and property. Therefore they were more likely to emphasize already existing ties of kinship or to create new ones by marriage. Such a habit already existed in France before the eighteenth century (Sabean 1976). This quickly led to the emergence of social classes in Nouvelle-France: the seigneurs, nobles, and rich merchants on the one hand and the commoners on the other hand. Because marriage is a key institution in social reproduction (Bourdieu 1976), a relationship between class and kinship rapidly developed among the settlers of Nouvelle-France (Molloy 1990).

Two of the first seigneuries to be established were awarded to Robert Giffard and his friends, Jean and Nol Juchereau, all from Tourouvre. They were successful in attracting several men from Mortagne, many of whom migrated to Nouvelle-France with their wives and children. In 1635 settlers from Perche constituted more than one-third of the 150 French Canadians. In fact, more than 75% of the migrants from Perche settled before 1654, when Nouvelle-France had only a few hundred inhabitants. Between 1608 and 1639 the Perche settlers represented 30.1% of the settlers in Nouvelle-France. Their contribution to the settling contingent decreased quickly to 12.7% between 1640 and 1659 and to less than 1% between 1660 and 1759 (Charbonneau 1970).

After the early years, when men by far outnumbered women, and the period of intense immigration in the 1660s, the population grew mainly by natural increase at an average rate of 25 per 1000. In the eighteenth century the death rate was 30 per 1000 and the birth rate was 55 per 1000. With these rates the French Canadian population would double in just under 30 years (Charbonneau and Harris 1987). By 1760 the 8500 settlers over the previous 150 years had become a population of 76,000 individuals.

The immigration from France stopped after 1760, and the population continued expanding quickly by natural increase. There was little admixture between the English, of Protestant faith, and the French Canadians, of Catholic faith. Today, an estimated 5 million inhabitants in Quebec are believed to be the descendants of the 8500 settlers established in the St. Lawrence River valley in the seventeenth and eighteenth centuries.

Dynamics of the Constitution of the Genetic Pool.

Twenty-four of the 28 hereditary disorders studied cluster in eastern Quebec (De Braekeleer and Dao 1994). One possible explanation is that most of these disorders were brought to Nouvelle-France by the early founders from Perche, most of whom settled in eastern Quebec.

Only 48 of the 217 settlers from Perche were common founders in at least one hereditary disorder, and 34 of them have been found in more than one disorder (De Braekeleer and Dao 1994). Therefore the last question to be answered is, How could so few individuals play such a major role? Part of the answer has already been given (large number of descendants). To better characterize the founding set in the hereditary disorders, we reconstructed the descending pedigrees of the founders from Perche who settled in eastern Quebec and appeared in more than one disorder and compared them with the pedigrees of those who did not appear as common founders in the disorders. These pedigrees were traced using Pedigree/Draw (version 4.3) provided by Mamelka (1991); the pedigrees spanned a time period from the early 1600s to 1730. Obviously, because of space availability, they are not illustrated here (more than 90 pages in reduced format). The most striking feature is that all the individual pedigrees can be assembled into two large but quite different pedigrees (Table 2). (Table 2 omitted.)

The first pedigree, referred to as the pedigree of the seigneurs, is arranged around the Giffard and Juchereau families. Affinity and kinship already existed in Mortagne between the Giffards and the Juchereaus. Intermarriages between their descendants were frequent during the first 100 years of the seigneurial system.

The second pedigree, called the pedigree of the commoners, is arranged around the Guyon and Cloutier families, both of which were engaged by and arrived with Robert Giffard in spring 1634. Thirty-eight surnames were repeatedly found in this pedigree. They represent 52 settlers, because in several instances multiple members of the same family migrated to Nouvelle-France. If the children arrived with both parents, only the parents were counted as settlers. Contrary to the individual nature of the settlers identified in the pedigree of the seigneurs, nuclear families of commoners moved to Nouvelle-France. In 12 cases the parents moved with their children.

This kin migration to Nouvelle-France is well illustrated in Figure 4. (Figure 4 omitted.) Although two genealogical links in sixteenth-century France could not be confirmed (identified by a question mark in the pedigree), Figure 4 shows that an extensive kin and affinity network already existed in Mortagne and in neighboring Maine. All the settlers (filled symbols in Figure 4) came from Mortagne, except individuals V16-V18, who were from St. Cosme-en-Vairais in Maine (see Figure 2 for localization). There were 3 sibships (individuals IV11-IV14, V13-V15, and V16-V18) accounting for 10 of the 23 settlers, whereas more remote relationships were found among most of the other settlers (e.g., individuals V4 and VI1, V11 and V12).

Although the places of origin in France of the commoners were as diversified as those of the seigneurs, a larger proportion of the commoners came from Perche, more precisely from Mortagne (71.4% vs. 18.2%).

The kin and affinity network that existed in France between the commoners got even tighter in Nouvelle-France through nonrandom matings. Differences were also noted in the number of consanguineous marriages. In the seventeenth and eighteenth centuries the Catholic church forbade marriage between kin related as third-degree cousins or closer (Moroni 1961; De Braekeleer 1991b). Dispensations could be bought if a justification for the marriage could be shown. However, the costs were set so high (to discourage consanguineous marriages) that only the wealthier families could afford them (Leclerc 1959b; Greer 1985). Furthermore, the cost that a family had to pay for the dispensation also depended on the level of relationship between the spouses. The seigneurs used the consanguineous marriages as part of a strategy to keep the properties in their families but also to maintain them as a social class with all their privileges. Because of the small availability of individuals to be married, the gap between social classes, and the patterns of intermarriage between families, the number of consanguineous marriages was expected to rise among the commoners. Indeed, the number of marriages between kin started to increase after 1720, at a time when the spouses were related at a level that required either a financially affordable dispensation or no dispensation at all (Molloy 1990; Morissette 1991).

Differences also existed in the proportion of descendants who became nuns or priests. The proportion of members of religious orders was much lower among the commoners than among the seigneurs (1.2% vs. 6%). As shown in Figure 5, the individuals belonging to a religious order were not randomly distributed in the families. (Figure 5 omitted.) Members of religious orders were usually recruited from people of noble birth in France before the revolution; therefore this custom apparently applied in Nouvelle-France. In both classes the consequence was the withdrawal of several women from marriage eligibility at a time when they were already by far outnumbered by men. This situation contributed, in part, to the dense kin networks.

No explanation was found to explain the higher proportion of prereproductive deaths among the seigneurs than among the commoners (28.7% vs. 20.1%), although an underreporting bias is possible. More commoners lived in rural areas, some of them remote, that were visited occasionally by the priests who were in charge of keeping the civic records. Therefore some early deaths may not have been reported to the priests, leading to an underestimation of the prereproductive mortality among the commoners. If the difference is not explained by an observation bias, then the consequence is a differential probability of gene transmission between the seigneurs and the commoners. The higher prereproductive mortality rate among the seigneurs also led to a lower number of descendants up to the end of 1729 than that among the commoners.

Indeed, there was a major difference in the mean number of descendants by settler between the commoners and the seigneurs (73 vs. 20.7 descendants). The result is a much higher probability of transmission of rare genes among the commoners than among the seigneurs. The commoners are also expected to have a higher genetic contribution to the French Canadian genetic pool than the seigneurs.

The distributions of the birthplaces of the descendants of the commoners and the seigneurs were different. The descendants of the seigneurs were more likely to be born in urban areas (78.7% vs. 22.7%). More descendants of the seigneurs were also born in the regions located west of Quebec City (42.1% vs. 9.6%), whereas the birthplaces of the descendants of the commoners clustered on the north and south shores of the St. Lawrence River, east of Quebec City (67.5% vs. 12.2%). Furthermore, among the commoners who were born in the regions east of Quebec City, there was a regional distribution of surnames. Some surnames (e.g., Tremblay, Bouchard, Racine, and Simard) were more likely to be found on the north shore; other surnames (e.g., Miville, Pelletier, and Caron) were more likely on the south shore, and still other sumames (e.g., Pouliot and Drouin) were more likely in the Ile d’Orleans. Some surnames (e.g., Guyon, Gagnon, Gagne, and Cote) were distributed in the several regions of eastern Quebec without apparent clustering in a particular region. However, among the surnames that were preferentially found on the north shore, differences existed in the surname distribution between Cote de Beaupre (e.g., Cauchon, Gravel, and Racine) and Charlevoix (e.g., Tremblay, Simard, and Bouchard). Therefore it appears that the surname distribution in the French Canadian population that is still observed among the regions of Quebec (Bouchard et al. 1985; De Braekeleer 1990) dates back to the early settlement of Nouvelle-France. Most of the hereditary disorders identified so far cluster in northeastern Quebec, more particularly in Charlevoix and in the two regions that were initially settled by inhabitants from Charlevoix (Saguenay-Lac-St.-Jean and Cote Nord) (De Braekeleer and Dao 1994). Therefore it is likely that most of these genes were brought by settlers established on the north shore.

Only 2 settlers in the pedigree of the seigneurs were identified as common founders in at least one hereditary disorder compared with 28 in the pedigree of the commoners (De Braekeleer and Dao 1994).

One has to be reminded that there were some limitations in building the pedigree of the commoners. First, the pedigree was assembled around only two early settlers (Jean Guyon and Zacharie Cloutier). Second, all the calculations were based on the settlers carrying a surname that was repeatedly found in the pedigree. Because the surnames follow a patrilineal pattern, settlers who had no sons or only a few of them were not repeatedly found and therefore were not counted. This is the case for Abraham Martin and Marguerite Langlois, who had nine children, six daughters who married (one to a settler and one to a son of Zacharie Cloutier) and three sons who did not marry. Jean Emard and Marie Bineau had three daughters who married (one to a son of Zacharie Cloutier) and no sons. Some of the children of these two couples married into the pedigree of the commoners and were identified as common founders in more than one hereditary disorder.

The comparison of both pedigrees showed that major differences exist between the seigneurs and the commoners (origin in France, place of settlement in Nouvelle-France, number of descendants, number of members of religious orders, prereproductive mortality, consanguinity). Intermarriage (including sibship exchange) between families was a consistent pattern in both pedigrees, however. Figures 6 to 9 show some examples of kin network through marriages. (Figures 6 to 9 omitted.)

In Figure 6 the woman II2 and her daughter III2 from a previous marriage married two brothers, II1 and III1. In Figure 7 sibship exchanges occurred between three families. Each time, two sibs married two sibs of another family. Figure 8 shows more examples of sibship exchanges also involving remarriages. Four Pouliot sibs (III1, III13, III11, III13) married four Chabot sibs. One of them (III13), remarried a Manseau woman (III14) whose brother (III15) married a fifth Pouliot sib (III16). Two more Pouliot sibs (III7 and III9) married two Audet sibs (III6 and III8), and the woman III4, widow of a Pouliot sib (III3), remarried a third Audet sib (III5).

Figure 9 shows a more extensive pedigree in which several families intermarried in three generations. These patterns of marriage, which occurred frequently from the origins of the French colony up to the 1950s in some regions of Quebec (Molloy 1990; Collard 1991; Barthelemi De Saizieu 1986; Garneau 1988), were repeated over and over in the pedigree of the commoners.

As expected from the works by Bourdieu (1976), Dechene (1974), and Sabean (1976), little admixture was observed between both pedigrees representing the two major social classes in seventeenth-and eighteenth-century Nouvelle-France. Because there was little admixture, these two social classes have presumably evolved independently (genetic drift).

Design of a Model.

Given the data and their interpretation and the results from our previous article (De Braekeleer and Dao 1994), a probable scenario explaining the presence and the distribution of the hereditary disorders in the French Canadian population can be elaborated. Most of the hereditary disorders were brought by a few migrants from Perche in the seventeenth century. These farmers and artisans settled in Quebec City and in the areas located east of Quebec City on both shores of the St. Lawrence River. Quickly, they engaged in sibship exchanges (nonrandom matings) with other families, in limited number, who belonged to the same social class and also came from Perche or from a few other French provinces. An alternative scenario is that the hereditary disorders, or at least some of them, were introduced into the French Canadian population by early migrants from provinces other than Perche who married into the families originating from Perche.

A close and dense kin network developed between the families, some of whom were already related in France. Because of a high fertility rate, rare genes were likely to be retained in the population and transmitted over the generations. By 1730 a geographic split had already occurred among the families, some settling the south shore, others the north shore, and the remaining families occupying both shores. In part because of the seigneurial system, which led to the development of the towns and villages, the families who settled in a particular area must have functioned in an autarkic manner with high endogamy. By chance, some genes of hereditary disorders were distributed on the north shore and others on the south shore.

Settlements spread along the St. Lawrence River, and this progression was marked by the establishment of parishes farther and farther away from Quebec City. The newly established parishes were settled, at least at their beginnings, mostly by kin-related individuals (usually whole families and/or sibs) who came from nearby regions [see, for example, Jette et al. (1991)].

In the nineteenth century, other regions not located along the St. Lawrence River, such as Saguenay-Lac-St.-Jean, were opened to settlement. Again, the initial settlement was made by kin-related migrants coming from the closest regions [see, for example, Gauvreau et al. (1991) and De Braekeleer (1991a)]. The small number of founders, their concentration in some specific regions of Nouvelle-France, the nonrandom matings, and the high endogamy followed by kin migration to adjacent regions newly opened to settlement explain why, even today, the hereditary disorders cluster in eastern Quebec and more particularly north of the St. Lawrence River (Charlevoix, Saguenay-Lac-St.-Jean, Cote Nord) (De Braekeleer 1991a; De Braekeleer and Dao 1994).

At the end of the nineteenth century and during the twentieth century, industrialization and changes in the economy induced further interregional migrations, leading to the dispersion of the hereditary disorders in Quebec (De Braekeleer et al. 1992; Tremblay-Tymczuk et al. 1992).

Most of the hereditary disorders cluster in northeastern Quebec, where the majority of migrants from Perche and their descendants settled in the seventeenth, eighteenth, and nineteenth centuries. A minority of them settled the south shore of the St. Lawrence River, east of Quebec City, where some hereditary disorders are also more prevalent. It is probable that the lower number of hereditary disorders clustering in western Quebec is the result of more diverse immigration to the French colony and greater population flow.

In summary, the presence and the high frequency of most of the hereditary disorders in the French Canadian population appear to be the result of founder effect and genetic drift.

ACKNOWLEDGMENTS–We are grateful to Jean-Franois Moreau for his suggestions and criticisms, which helped to improve this manuscript. This research was supported in part by a grant from the Fondation de l’universite du Quebec a Chicoutimi.

FOOTNOTES

1. Research Laboratory in Genetic Epidemiology, Departement des Sciences Humaines, Universite du Quebec a Chicoutimi, 555 Boulevard de l’Universite, Chicoutimi, Quebec G7H 2B1, Canada.

2. Insitut National d’Etudes Demographiques, 27 rue du Commandeur, 75675 Paris, France. Address correspondence to M. De Braekeleer at his French address.

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