On the perpetuation of relic genes having an inviable homozygote

Reproductive compensation may be prezygotic or postzygotic. Prezygotic compensation is the replacement of inviable infants by one or more additional births. When the marital fertility rates are greater than necessary to replace the parents, however, many traditional societies ensured that any surviving progeny in excess of two (on average) did not marry, or married only if they replaced an older sib as heir to the family estate. We call this postzygotic compensation. We show that the coefficient of biologically effective compensation approximates unity when normal and genetic-disease-affected sibships were both sufficiently large to ensure that, on average, two or more sibs survived to the end of the reproductive period, irrespective of the level of prezygotic compensation. The impact of these types of compensation on the population genetics of Tay-Sachs disease and cystic fibrosis is discussed.