Advances in Anthropology
2012. Vol.2, No.3, 132-138
Published Online August 2012 in SciRes (http://www.SciRP.org/journal/aa) http://dx.doi.org/10.4236/aa.2012.23016
Copyright © 2012 SciRe s .
132
Tay-Sachs and French Canadians: A Case of Gene-Culture
Co-Evolution?
Peter Frost
Bernard Saladin d’Anglure, Anthropology Departm e nt, Université Laval, Quebec City, Canada
Received May 10th, 2012; revised June 15th, 2012; accepted June 27th, 2012
Tay-Sachs, an inherited neurological disorder, is unusually common among French Canadians from east-
ern Quebec. Two alleles are responsible, one being specific to the north shore of the St. Lawrence and the
other to the south shore. This pattern of convergent evolution suggests the presence of a selection pressure
limited to eastern Quebec. Both alleles probably arose after the British conquest of Quebec in 1759 or at
least were uncommon previously. To explain the high incidence of Tay-Sachs among Ashkenazi Jews,
some authors have invoked heterozygote advantage, i.e., heterozygous individuals enjoy a higher rate of
neuronal growth, and thus greater learning capacity, without the neurological deterioration of homozy-
gous individuals. Such an advantage would have helped Ashkenazim perform the mental effort required
for work in trade and crafts. A similar situation may have developed in eastern Quebec, where the relative
scarcity of British and American merchants made it easier for French Canadians to enter occupations that
required literacy, numeracy, and future time orientation.
Keywords: Neurological Disorder
Introduction
Tay-Sachs is an inherited neurological disorder caused by
defective lysosomal storage in neural tissue. The earliest sign is
usually an exaggerated startle response. Psychomotor deteriora-
tion appears around 4 to 6 months of age, followed by axial hy-
potonia, limb spasticity, seizures, and blindness. Progressive
neurological deterioration leads to death by the age of four.
Tay-Sachs is common among Ashkenazi Jews of Eastern
European origin and is often thought of as a Jewish disease. Yet
it also reaches high incidences in a few other populations, par-
ticularly French Canadians.
Among French Canadians, it has three unusual characteris-
tics:
1) It is highly localized geographically, being concentrated in
eastern Quebec (Figure 1). In Rimouski, the heterozygote fre-
quency is 7.6%, versus 4.2% among Ashkenazi Jews and 0.3%
among French Canadians in Montreal (De Braekeleer et al.,
1992).
2) It is caused by two mutant alleles: one that arose on the
north shore of the St. Lawrence (regions of Charlevoix, Sague-
nay, Lac Saint-Jean) and another that arose on the south shore
(Bas Saint-Laurent) (Hechtman et al., 1992; Zlotogora, 1994;
De Braekeleer, 1995).
3) It seems to be relatively recent in origin, these two alleles
being absent in France. Both probably arose after the British
conquest of Quebec in 1759 or at least were uncommon previ-
ously (Hechtman et al., 1992; De Braekeleer, 1995).
These characteristics are inconsistent with a founder effect,
i.e., a chance deviation in gene frequency between a population
and its parental stock. A founder effect might explain French
Canadian Tay-Sachs if only one mutation were the cause. Here,
two normally rare mutations, with the same physiologica l effec t,
have become prevalent within the same culture area in adjacent
populations. This looks more like convergent evolution of two
populations through a shared pressure of natural selection.
Founder Effect?
Yet the literature routinely explains French Canadian Tay-
Sachs as a founder effect. This is largely because eastern Que-
bec is home to other genetic disorders with similarly high inci-
dences. There is thus a pattern of apparent founder effects that
might reflect the area’s ethnic homogeneity and historically fast
population growth.
These other eastern Quebec disorders nonetheless resemble
Tay-Sachs in one respect. Of the top ten, six are primarily neu-
rological and two secondarily so (Table 1; Laberge et al., 2005).
Is this pattern typical? Of the top ten genetic disorders in the
United Kingdom, only three are primarily or secondarily neu-
rological (Table 2) (Genetic Alliance UK, 2010).
One of the eastern Quebec disorders, mucolipidosis type II,
is a lysosomal storage disease like Tay-Sachs. It reaches a het-
erozygote frequency of 2.6% in the regions of Saguenay and
Lac Saint-Jean, the highest reported rate for this disorder
(Plante et al., 2008). French Canadians are also thought to have
high incidences of two other lysosomal storage diseases: G au c h er ’ s
disease and mucopolysaccharidosis type IV (Laberge et al.,
2005).
Natural Selection?
The alternate explanation, natural selection, implies that car-
riers of Tay-Sachs enjoy an advantage over non-carriers, spe-
cifically a reproductive one. Such an advantage may seem un-
likely, since Tay-Sachs leads to early death and no reproduction.
But this outcome happens only if the carrier has both copies of
a Tay-Sachs allele (homozy gote) and not just one (heterozy gote).

P. FROST
Figure 1.
Areas of high Tay-Sachs prevalence in Eastern Queb e c.
Table 1.
Genetic disorders with higher incidences in Eastern Quebec.
Genetic disorder Location Heterozygote
frequency Symptoms
Tay-Sachs—south-shore variant Bas Saint-Laurent 7.6% Startle response, psychomotor deterioration, axial hypotonia, limb
spasticity
Tay-Sac hs—north-shore varian t Charlevoix/Sa guenay/Lac
Saint-Jean 1.1% As above
Mucolipidosis type II Saguenay/Lac Saint-Jean 2.6% Delayed development with impacts on organ size, skeletal growth,
and motor skills
Autosomal recessive spastic ataxia of
Charlevoix-Saguenay Saguenay 4.5% Polyneur opathy b y third d ecade of li fe, sever e denerv ation, slo wer motor nerve c onduction, absence of sensory action potentials
Agenesis of corpus callosum and
peripheral neuropathy Saguenay 4.3% Progressive axonal degeneration with perip heral neuropathy
Leigh syndrome, French Canadian type Saguenay/Lac Saint-Jean Movement disorders, developmental delay, hypotonia, high lactate
levels in blood and cerebrospinal fluid, high mortality in infancy
from acute acidotic and/or neurological episodes
Jumping Frenc hmen of Maine Beauce Startle responses: jumping, raising of arms, shouting, hitting,
repeating of sentences
Tyrosinem ia type I Saguenay/Lac Saint-Jean 4.5% Cirrhosis, he patocellular carcinoma, renal tubulopathy with
hypophospha temic rickets a nd mild renal Fanconi syndrome, acute
neurological crises with se v ere pain in the extremities and
sometimes
p
aral
y
sis
Pseudo-vitamin D-deficiency rickets Charlevoix/Saguenay/Lac
Saint Jean 3.7% Rickets, hypocalcemia, hypophosphatemia, secondary hyperparathyroidism
Cystinosis Saguenay/Lac Saint Jean 2.6% Ocular photosensitivity, ricke t s, proximal renal tubular acidosis,
progressive renal insufficiency
Sources: Laberge et al. (2005); Braekeleer (1995).
Because the second situation is much more common than the
first, the slight advantage of having one copy could offset the
great disadvantage of having two.
This argument has been used to explain why Ashkenazi Jews
have high incidences of four lysosomal storage disorders:
Tay-Sachs, Gaucher’s, Niemann-Pick, and mucolipidosis type
storage by lysosomes of sphingolipids, a substance that pro-
motes growth of neural axons and that may thus increase learn-
ing capacity. It is perhaps for this reason that engineers and
scientists are at least six times more common among Israelis
with Gaucher’s disease than among Ashkenazi Jews in general.
Heterozygous individuals should have modestly higher sphin-
IV (Cochran et al., 2006). These disorders lead to excessive golipid storage and a correspondingly higher IQ, “probably in
Copyright © 2012 SciRe s . 133

P. FROST
Table 2.
Top ten genetic disorders in the United Kingdom.
Genetic disorder Incidence (per 1000 births) Symptoms
Familial combined hyperlipidemia terol level s , l eading to hea rt di s ease 5.0 High choles
Familial hypercholesterolemia 2.0 High cholesterol levels, leading to heart disease
Dominant oto sclerosis 1.0 Abnormal bone growth near the mi dd le ear
Adult polycystic kidney dise ase
lop on the s keleton
ination, cognitive
ue
juices, and mucus
ular dystrophy
0.8 Cysts that de stroy the kidney
Multiple e x o stoses 0.5 Bony spurs or lumps that deve
Huntington’s dise as e0.5 Neurodegeneration, leading to loss of muscle coord
decline, and dementia
Fragile X syndrome 0.5 Autism and intellectual disability
Neurofibromatosis 0.4 Tumors that devel op out of nerve tiss
Cystic fibrosis 0.4 Defective production of sweat, digestive
Duchenne musc0.3 Muscle dege neration
S).
n et al., 2006: p. 677). Ashkenazi
da: Historical and
Economic Background
Is?
The idea might ch Canadians
hi
r numbers were constrained by cultural
an
e (1608-1759)
market as a very secondary
the countryside, where three-
qu
Government officials controlled food prices and wholesale
trade (Vallières et al., 2008: pp. 219, 223, 520, 521). To give
hants were forbidden to purchase
fr
noted by the Jesuit missionary and explorer Pierre-
Fr
need… (Ouellet, 1966: p. 7).
a
e requisites of their own consumption
an
n the road to a
more market-driven economy. Great Britain itself was moving
advocacy of free trade
ore local level, the con-
ource: Genetic Alliance UK (2010
the order of 5 points” (Cochra
This neural advantage should have helped many
Jews adapt to tasks that require literacy, numeracy, and future
time orientation, notably the buying and selling of goods and
the making of crafts for urban markets (Cochran et al., 2006;
Murray, 2007). The Tay-Sachs allele would have thus become
more common, since the more successful traders and artisans
contributed disproportionately to population growth, particu-
larly the surge from about 100,000 Ashkenazim in 1650 to 5
million in 1900 (Risch et al., 1995).
Tay-Sachs in French Cana
Tay-Sachs common in eastern Quebec for the same reason
seem farfetched. Were not Fren
storically a nation of farmers? And were not English Canadi-
ans the businessmen?
In reality, there had always been some French Canadian
businessmen, but thei
d even legal barriers or by competition from British and
American immigrants. These constraints varied in strength over
time and space. Whereas cultural and legal barriers were
stronger under the French Regime, immigrant competition was
stronger under the British Regime, especially in western and
central Quebec.
French Regim
The French colonists saw the
source of the necessities of life. In
arters of them lived, almost all food, clothing, and furnish-
ings were produced within the family. Although people also
worked outside the family, such labor was usually not bought
or sold but instead supplied under long-term agreements of
mutual assistance, such as between tenant farmers and their
seigneur. Farming itself was primarily for subsistence and only
secondarily for sale of surplus produce to middlemen. Wheat
yields were only 4 to 6 bushels per arpent (0.85 acre) (Ouellet,
1966: pp. 8-9, 52-53, 81-82).
In the towns, markets did exist but were State-regulated.
consumers first priority, merc
om farmers directly and before certain hours of the morning
(Vallières et al., 2008: p. 505). Urban markets were localized in
space and time, being typically marketplaces where buyer and
seller met face to face and personally supervised each transac-
tion. These oases of trade could not coalesce into a true market
economy largely because buyers and sellers trusted each other
so little.
If lack of trust confined the market economy to isolated
points in space, lack of foresight confined it to the present point
of time. People tended to sacrifice future needs for short-term
gains, as
ançois-Xavier de Charlevoix:
The English colonist amasses worldly goods and makes no
superfluous expenses; the French colonist enjoys what he has,
and often shows off what he has not. The former works for his
heirs; the latter leaves his own in
This was one reason why the French government distrusted
the market mechanism and felt obliged to act on behalf of
long-term interests. After the conquest, the first British gover-
nor felt similarly, seeing therein an obstacle to development of
true market economy:
… the inhabitants are inclinable enough to be lazy, and not
much skilled in Husbandry. The great dependancies they have
hitherto had on the Gun and fishing rod, (which) made them
neglect tillage beyond th
d the few purchases they needed, the Monopolies that were
carried on here in every branch, (which) made them careless of
acquiring beyond the present use, and their being often sent on
distant parties and detachments, to serve the particular pur-
poses of greedy and avaricious men without the least view to
public utility, were circumstances under which no country
could thrive (Murray, 1902 (1762): pp. 51-52).
British Regime and after (1759-)
The British conquest of 1759 put Quebec o
in the same direction with its growing
and rejection of mercantilism. On a m
Copyright © 2012 SciRe s . 134

P. FROST
qu
s of its values. This was per-
ha
ships. A similar situation prevailed on the south-
er
this
te
e does not hear English at all.
Th
er, closed shop after a few years. Only those
po
special talents. This point is made in a bi-
og
erchant is not pos-
si
t in John Guay’s case. The
fa
egan as innkeepers, as tav-
er
been dis-
cu
mber and re-
eventu-
s. Their
va
families (Seccombe, 1992: pp.
18
y while keeping the actual production process in a
pr
est weakened groups that previously had State backing,
thereby freeing up room for merchants, petty traders, self-em-
ployed artisans, and other entrepreneurs who could navigate in
the new economic environment.
A gulf developed between the landed gentry, who thought of
society in terms of absolutism, feudalism, bureaucracy, birth,
and tradition, and the middle class (bourgeoisie) who now in-
tended to redefine society in term
ps one of the most important consequences of the conquest.
The bureaucracy as well as the landed gentry and the military
elite saw their social role challenged by a middle class that
demanded its autonomy and that intended to exercise a social
influence in proportion to its economic dynamism (Ouellet,
1966: p. 96).
This ascendant class was filled by British and American im-
migrants in western and central Quebec, particularly in the
Ottawa Valley, Montreal, Trois-Rivières, Quebec City, and the
Eastern Town
n and eastern shores of the Gaspé Peninsula (Figure 1).
In contrast, such immigrants were scarce over much of east-
ern Quebec, i.e., the Charlevoix, Saguenay, and Lac Saint-Jean
regions on the north shore of the St. Lawrence and the Beauce
and Bas Saint-Laurent regions on the south shore. Since
rritory lay farther from the American border and lacked major
ports, it became home overwhelmingly to local settlers of
French descent. Nonetheless, its built environment—roads,
bridges, buildings, etc.—was almost wholly British in design
because settlement was largely post-conquest. Social structures
were likewise less influenced by the French Regime. The sei-
gneurial system lasted only a few decades in Bas Saint-Laurent
versus nearly two centuries farther upriver (Fortin & Lechas-
seur, 1993: p. 311).
The absence of English-speaking immigrants was striking to
Alexis de Tocqueville when he passed through Bas Saint-
Laurent in 1831:
In this portion of Canada, on
e population is only French, and yet when one encounters an
inn, or a merchant, the sign is in English (De Tocqueville, 2003
(1831), p. 185).
With little competition from British or American merchants,
an enviable niche opened up for French Canadians, or rather for
those with the right abilities. In the Saguenay region during the
19th century:
Success in trade was never easy because of the competition,
the fragility of the markets, and the instability of business con-
ditions. The ablest managed to live or survive. Others, the
greatest numb
ssessing exceptional qualities would make a fortune (La-
pointe, 1996: p. 3).
Likewise in the Beauce region, “out of ten merchants who
remained more than ten years in business, only four or five
succeeded” (Courville et al., 2003: p. 257).
Success required
raphy of John Guay (1828-1880), a leading French Canadian
merchant of the Saguenay region:
Improvising one’s way into becoming a m
ble for anyone who so wishes. It takes talent and of course
capital. Spontaneous generation was very uncommon in 19th-
century Quebec, indeed nonexisten
mily environment in which he grew up predisposed him to
develop an interest and also aptitudes for the businesses of
trade, forestry, and farming. His successful career henceforth
proved that a French Canadian merchant could ably penetrate
the world of business; a world where, let us remember, English
Canadians controlled most of the commercial and industrial
activities (Lapointe, 1996: p. 126).
How did French Canadians penetrate the world of business?
John Guay’s father built bridges, wharfs, and sawmills on con-
tract before becoming a merchant (Lapointe, 1996: pp. 18-24).
In the Beauce region, such people b
n operators, or as farmers who worked on the side as live-
stock or grain dealers (Bélanger et al., 1990: p. 62; Courville et
al., 2003: pp. 261-262). Often, the initial line of work would
never be fully given up, a reason perhaps why so many French
Canadian merchants of the 19th century failed to identify
themselves as such to census takers (Courville et al., 2003: pp.
261-262). It may also be that the title of marchand or com-
merçant was viewed as somewhat disreputable.
To become a full-fledged merchant, the preconditions seem
to have been familiarity with cash transactions and frequent
negotiation of agreements on a case-by-case basis. A third one
was a certain outlook on life. This precondition has
ssed by the historical economist Gregory Clark with respect
to England from the 12th century onward. As England became
a settled society, with the State imposing a monopoly on vio-
lence, successful individuals were those who could settle dis-
putes amiably and profit from current actions further into the
future—in short, those who had middle-class values of thrift,
foresight, self-control, and sobriety (Clark, 2007).
Expansion of a M arket-Orient ed Middle Class
Such individuals were initially a small minority in England.
Their descendants, however, steadily grew in nu
placed the lower classes through downward mobility,
ally accounting for most of the population by the 1800
lues came to dominate English society and laid the moral,
cultural, and behavioral basis for the Industrial Revolution
(Clark, 2007, 2009a, 2009b).
The Industrial Revolution would end this process of demo-
graphic change. Previously, production took place in family-run
cottage industries whose owners, if successful, expanded their
workforces by having bigger
2-183, 205-206). Now, production was in factories whose
owners simply hired as many workers as they wished. These
new industrialists did not translate their economic success into
reproductive success. Henry Ford, for instance, had only one
child.
In Quebec, the family remained the most common unit of
production well into the 20th century. This was especially so on
farms and in small businesses that dealt directly with the market
econom
e-market economy of unpaid family labor. There was thus a
strong incentive to have children. In Quebec City, at the turn of
the 20th century, the fertility rate was higher among French
Canadians who worked for themselves at home than among
those who worked for an employer at another location. A
home-based shoemaker could bring his wife and children into
different stages of production, i.e., cutting the hides, dyeing,
sewing, fitting pieces together, etc. This family assistance was
unavailable to wage-earning factory employees (Marcoux et al.,
2006: pp. 73-85; Marcoux, 2009: pp. 107-120).
Like crafts men and women, merchants had access to child
labor. In addition, they could earn much more than the average
Copyright © 2012 SciRe s . 135

P. FROST
French Canadian and thus benefit from a positive feedback
between family size and income. The merchant John Guay had
te
. Whatever the
lin
family formation, being better able
to
s—created a distinct cultural environment with
ed in this environment, one had to
e had to adapt to different climates,
la
sired behavior through conscious
ef
a new cultural environment, which in
tu
rt.
and other disorders in eastern Quebec.
Su larger number of gener-
al
es-
tions.
W
icant genetic change over a span of two and a
half centuries? This is a mere ten generations. Yet the time span
ter. Given that the two Tay-
Sachs mutations
erican border. Like
the rest of eastern Quebec, it has had relatively little settlement
d has thus developed its own middle
cl
n children who lived to adulthood, twice the average of Fre nch
Canadians of his time (Lapointe, 1996: p. 110).
In all this, what mattered was not so much one’s occupation
per se as one’s access to the market economy, a factor that
varied within each occupation. Farming, for instance, ranged
from subsistence agriculture to market gardening
e of work, by better exploiting the possibilities of the market
economy, one could generally earn more income and support
more children, who in turn could marry earlier and have larger
families of their own. More income also meant less mortality,
especially infant mortality—a major curb on reproductive suc-
cess until the 20th century.
This factor might explain why fertility correlated positively
with literacy in all occupations of the Saguenay region through-
out the 19th century and long after. Literate individuals may
have had more resources for
navigate in the market economy and needing less supervision
by third parties (employer, manager, custodian, etc.). This cor-
relation would not become negative until 1946-1955 among
farmers and 1931-1935 in other occupations, a reversal that
came much later than elsewhere apparently because of the Ro-
man Catholic Church’s hostility to contraception (Bouchard &
Roy, 1991).
Baldwinian Selection
The growing number of market-oriented i ndivi duals—in s h o rt,
the middle clas
its own norms. To succe
adapt, just as humans hav
ndscapes, and food sources.
Cultural evolution thus paved the way for biological evolu-
tion. This process, called Baldwinian selection, can be broken
down into three stages:
1) Individuals adh er e to a d e
fort, within an envelope of possibilities allowed by their ge-
netic endowment.
2) These actions create
rn selects for genotypes that more easily produce the desired
behavior. A heritable predisposition increasingly takes over
from conscious effo
3) The result is a shift toward a new mean genotype and a
new envelope of possible phenotypes.
This kind of gene-culture co-evolution may explain the high
incidence of Tay-Sachs
ch disorders would be only the tip of the iceberg, i.e., the
most harmful outcomes among a much
ly benign outcomes. We know less about the latter because
there is less interest in genetic traits that cause no harm.
Discussion
Have French Canadians been shaped by gene-culture co-
evolution? The possibility is intriguing but raises several qu
as There Enough Time?
One is the length of time. How could natural selection have
produced signif
could not have been much grea
are absent in France and uncommon over most
of Quebec, both alleles must have reached their currently high
prevalence in eastern Quebec after French Canadians had begun
to settle that area.
If the Tay-Sachs heterozygote frequency had been 0.3%
among French Canadians at the time of the conquest, ten gen-
erations could have been enough to bring it up to the current
level of 7.6% in Rimouski if we assume a 25% - 30% fitness
advantage. In addition, there may have been selection effects
from the mass out-migration of the late 19th century when
many French Canadians left the area for factory work in New
England. The emigrants tended to be landless farmers, rather
than merchants, and may have been less likely to carry the Tay-
Sachs allele.
Such rapid evolution seems to have been common in our
species. Natural selection has altered at least 7% of the human
genome over the last 40 thousand years, with most of the
change happening since agriculture emerged less than ten
thousand years ago. At that time, the rate of genetic change
may have increased over a hundred-fold (Hawks et al., 2007).
This rapid evolution seems to reflect rapid adaptation to a wide
range of new environments: not only physical environments
defined by climate, landscape, and vegetation, but also cultural
environments defined by technology, social structure, and codi-
fied rules of behavior.
This kind of gene-culture co-evolution has recently been
shown among French Canadians, specifically those inhabiting
the island of Île aux Coudres in the St. Lawrence. Its settlers
came from a land-poor environment where young people post-
poned marriage until their parents handed over the farm. Emi-
gration to New France, where farmland was much more plenti-
ful, dramatically lowered the age of marriage. This decline in
the age of marriage was followed by a decline in the mean age
of first reproduction (AFR). From 1800 to 1940, AFR fell by
four years. This second decline was due not to a lowering of the
mean age of marriage (now stable) but to a shortening of the
mean interval between marriage and first birth. The ultimate
cause could not have been a change in nutrition. Nor could it
have been some kind of cultural lag, since AFR fell at the same
rate throughout this period. The likeliest explanation is a grad-
ual shift in the gene pool due to a growing number of highly
fertile couples who could better exploit the opportunities for
early family format io n (Milot et al., 2011).
The Beauce Region
The Beauce region stretches southeast of Quebec City and
covers the Chaudière valley up to the Am
by English-speakers an
ass, so much so that Beaucerons are stereotyped as business-
minded go-getters—the “Yankees” of Quebec. Yet this same
region has low incidences of Tay-Sachs and also of most of the
other disorders that are frequent in eastern Quebec.
Something else, however, distinguishes the Beauce region
from the rest of eastern Quebec. Settlers arrived there earlier
than elsewhere. They also seem to have been less homogeneous,
coming from a wider range of regions in France and including
discharged German mercenaries (Ferron & Cliché, 1982: pp. 3-
4, 123-124). This larger and more diverse gene pool may have
supplied a greater number of suitable individuals for the niches
Copyright © 2012 SciRe s . 136

P. FROST
that opened up after the conquest, as Quebec moved from a
semi-feudal society to a market-driven one. Natural selection
had more leeway to favor new genetic variants that cause fewer
harmful side effects.
Tay-Sachs Elsewhere in North America
High incidences of Tay-Sachs have been reported from three
other groups in North America: the Cajuns of Louisiana; a
Pennsylvania Dutch community; and people of Irish origin
ell et al., 1992;
be a
fo
als tended to have a less opti-
m
ciency rickets and cystinosis. Both lead to rickets
th
lumes and show more cell proliferation throughout
th
., & Beauchet, O. (2009). Vitamin D and cognitive
performance in adults: A systematic review. European Journal of
Neurology, 16, 1083-1089. doi:10.1111/j.1468-1331.2009.02755.x
(Branda et al., 2004; Kelly et al., 1975; McDow
Mules et al., 1992). In the second group, the cause may
under effect, since there is no exclusive geographic or social
basis. The disease occurs within several related families who
form 3% of the population of two townships (Kelly et al., 1975).
On the other hand, two separate alleles are responsible, despite
the small size of this semi-isolate (Mules et al., 1992). A foun-
der effect less easily explains Tay-Sachs among the Cajuns and
the Irish, who have multiple Tay-Sachs alleles and form geo-
graphically or ethnically circumscribed groups (Branda et al.,
2004; McDowell et al., 1992).
The Cajuns and the Irish may have likewise gone through
this kind of gene-culture co-evolution. Like the French Cana-
dians of eastern Quebec, they made the transition to a market
economy later and more quickly than the English did. It may be
that their market-savvy individu
al mix of genetic factors, including alleles like Tay-Sachs that
are detrimental in homozygotes though useful in hetero z ygote s.
Why Are Vitamin-D Deficiency Disorders Also More
Prevalent?
Finally, although gene-culture co-evolution might explain
why certain neurological disorders occur more often in eastern
Quebec than elsewhere, it less easily explains the higher preva-
lence of two apparently non-neurological disorders: pseudo-
vitamin-D defi
rough an inability to synthesize or process enough vitamin D.
This symptom is also associated with a third eastern Quebec
disorder: Tyrosinemia type I. In comparison, rickets is not
caused by any of the top ten genetic disorders in the United
Kingdom.
Vitamin-D deficiency does not seem to affect adult cognition,
at least not according to a recent review article (Annweiler et al.,
2009). But there may be early developmental effects. When rat
fetuses are deprived of vitamin-D, the newborn pups have lar-
ger brain vo
eir brains. This is consistent with the antiproliferative effect
of this vitamin on body tissues. Prenatal vitamin-D deficiency
seems to increase the rate of neuronal proliferation while de-
creasing the rate of neuronal cell death (Eyles et al., 2003). If
prenatal vitamin-D deficiency affects humans similarly, the
result may be improved cognitive performance, albeit at a high
cost for homozygous individuals. As with Tay-Sachs, the more
numerous heterozygous individuals should enjoy a lower cost/
benefit ratio.
REFERENCES
Annweiler, C., Allali, G., Allain, P., Bridenbaugh, S., Schott, A.-M.,
Kressig, R. W
Bélanger, F., Berberi, r, D., & Lessard, R.
B
Br
S., Breton, J.-R., Carrie
(1990). La Beauce et les Beaucerons. Portraits d’une région
1737-1987. Saint-Joseph-de-Beauce: La Société du patrimoine des
Beaucerons.
ouchard, G., & Roy, R. (1991). Fécondité et alphabétisation au Sa-
guenay et au Québec (XIXe-XXe siècles). Annales de démographie
historique, 3, 173-201.
anda, K. J., Tomczak, J., & Natowicz, M. R. (2004). Heterozygosity
for Tay-Sachs and Sandhoff diseases in Non-Jewish Americans with
ancestry from Ireland, Great Britain, or Italy. Genetic Testing, 8,
174-180. doi:10.1089/gte.2004.8.174
lark, G. (2009a). The indicted and the wealthy: Surnames, reproduc-
tive success, genetic sele
C
C
C
ction and social class in pre-industrial Eng-
land.
http://www.econ.ucdavis.edu/faculty/gclark/Farewell%20to%20Alm
s/Clark%20-Surnames.pdf
lark, G. (2009b). The domestication oCf man: The social implications
of Darwin. ArtefaCTos, 2, 64-80.
lark, G. (2007). A farewell to alms. A brief economic history of the
world. Princeton: Princeton University Press.
ochran, G., Hardy, J., & Harpending, H. (2006). Natural history of
Ashkenazi intelligence. Journal of Bi osoci al Science, 38, 659-693.
doi:10.1017/S0021932005027069
ourville, S., Poulin, P. C., RodrCigue, B., Allard, M., Girard, D.,
Grenier, F., Hatvany, M., Larocque, M.-J., Mcquillan, A., Poulin, P.,
Pronovost, C., Routhier, G., Schwartz, K., & St-Hilaire, M. (2003).
Histoire de Beauce-Etchemin-Amiante. Quebec City: Presses de
l’Université Laval.
e Braekeleer, M. (1995). InbreedingD, kinship and surnames in heredi-
tary disorders: The experience in Saguenay-Lac-Saint-Jean (Quebec).
Collegium Antropologicum , 19, 289-304.
e Braekeleer, M., Hechtman, P., Andermann, E., & Kaplan, F. (1992).
The French Canadian Tay-Sachs disease deletion mutation: Identifi-
cation of probable fou
D
nders. Human Genetics, 89, 83-87.
doi:10.1007/BF00207048
e Tocqueville, A. (2003) [1831]. Regards sur le Bas-Canada. Mont-
real: Typo.
D
Eyles, D., Brown, J., Mackay-Sim, A., Mcgrath, J., & Feron, F. (2003).
Vitamin D3 and brain d e v e l o pment. Neuroscience, 118, 641-653.
doi:10.1016/S0306-4522(03)00040-X
erron, M., & Cliché, R. (19F82). Les Beaucerons. Ces insoumis, suivi
de Quand le peuple fait la loi. Montreal: Hurtubise HMH.
ortin, J.-C., & FLechasseur, A. (1993). Histoire du Bas-Saint-Laurent.
Gst
4, 20753-
HDe Braekeleer, M., Andermann, E., Melan-
Quebec City: Presses de l’Uni versité Laval.
enetic Alliance UK (2010). Incidence of genetic disorders, URL (la
checked 25 June 2012).
http://www.geneticalliance.org.uk/education3.htm
awks, J., Wang, E. T., Cochran, G. M., Harpending, H. C., & MHoyzis,
R. K. (2007). Recent acceleration of human adaptive evolution. Pro-
ceedings of the National Academy of Sciences of USA, 10
20758.
echtman, P., Boulay, B.,
çon, S., Larochelle, J., Prevost, C., & Kaplan, F. (1992). The intron 7
donor splice site transition: A second Tay-Sachs disease mutation in
French Canada. Human Genetics, 90, 402-406.
doi:10.1007/BF00220467
elly, T. EK., Chase, G. A., Kaback, M. M., Kumor, K., & McKusick, V.
A. (1975). Tay-Sachs disease: High gene frequency in a non-Jewish
population. American Journal of Human Genetic s , 27, 287-291.
aberge, A.-M., Michaud, J., Richter, A., Lemyre, E., Lambert, M.,
Brais, B., & Mitchell, G. A. (2005). Population hi
on medical genetics in Que
Lstory and its impact
bec. Clinical Genetics, 68, 287-301.
doi:10.1111/j.1399-0004.2005.00497.x
apointe, N. (1996). Le capitalisme marchand au Saguenay-Lac-Saint-
Jean: John Guay (1828-1880), négociant et propriétaire fon
L
Mier,
t modernités
M
cier.
Master’s Thesis, Quebec City: Université du Québec à Chicoutimi.
arcoux, R. (2009). Transition démographique et mise au travail des
enfants dans la ville de Québec à la fin du XIXe siècle. In B. Caul
& Y. Rousseau (Eds.), Temps, espace e (pp. 107-120).
Quebec City: Presses de l’Uni versité Laval.
arcoux, R., St. Hilaire, M., & Harton, M.-E. (2006). Modes de pro-
Copyright © 2012 SciRe s . 137

P. FROST
Copyright © 2012 SciRe s . 138
t
Mhapira, E., & Blitzer, M.
M to natural selec-
duction et comportements en matière de fécondité à Québec au débu
du 20e siècle: La nécessité de saisir le travail des femmes en milieu
urbain. Population et travail—Dynamique démographique et tra-
vail (pp. 73-85) . Actes des Colloques de l’AIDELF.
cDowell, G. A., Mules, E. H., Fabacher, P., S
G. (1992). The presence of two different infantile Tay-Sachs disease
mutations in a Cajun population. American Journal of Human Ge-
netics, 51, 1071-1077.
ilot, E., Mayer, F. M., Nussey, D. H., Boisvert, M., Pelletier, F., &
Réale, D. (2011). Evidence for evolution in response
tion in a contemporary human population, Proceedings of the Na-
tional Academy of Sciences of USA, 108, 17040-17045.
doi:10.1073/pnas.1104210108
ules, E. H., Hayflick, S.M, Dowling, C. E., Kelly, T. E., Akerman, B.
R., Gravel, R. A., & Thomas, G. H. (1992). Molecular basis of Hexo-
samininidase A deficiency and pseudodeficiency in the Berks County
Pennsylvania Dutch. Human Mutation, 1, 298-302.
doi:10.1002/humu.1380010406
urray, C. (2007). Jewis h ge ni us . MCommentary, April.
O
Pt, S., Roquis,
photrans-
Murray, J. (1902). [1762]. Report of the state of the government of
Quebec in Canada. Quebe c City: Dussault & Proulx.
uellet, F. (1966). Histoire économique et sociale du Québec 1760-
1850, structures et conjoncture. Fides: Montreal and Paris.
lante, M., Claveau, S., Lepage, P., Lavoie, E.-M., Brune
D., Morin, C., Vézina, H., & Laprise, C. (2008). Mucolipidosis II: A
single causal mutation in the N-acetylglucosamine-1-phos
ferase gene (GNPTAB) in a French Canadian founder population.
Clinical Genetics, 73, 236-244.
doi:10.1111/j.1399-0004.2007.00954.x
isch, N., De Leon, D., Oz elius, L. , Kra mer, P., Almasy, L ., Sing er, B.,
Fahn, S., Breakefield, X., & Bress
idiopathic torsion dystonia in Ashkenaz
Rman, S. (1995). Genetic analysis of
i Jews and their recent de-
scent from a small founder population. N a tu r e G e n et i cs , 9, 152- 159.
doi:10.1038/ng0295-152
eccombe, W. (1992). A millennium of family change. Feudalism to
capitalism in Northwestern Europe. Lond on: Ve rso.
allières, M., Desloges, Y.,
S
V Harvey, F., Heroux, A., Auger, R., La-
City: Presses de
Z
10-13. doi:10.1002/ajmg.1320490104
montagne, S.-L., & Charbonneau, A. (2008). Histoire de Québec et
de sa région. Tome I. Des origines à 1791. Quebec
l’Université Laval.
lotogora, J. (1994). High frequencies of human genetic diseases:
Founder effect with genetic drift or selection? American Journal of
Medical Genetics, 49,