Royal dynasties as human inbreeding laboratories: the Habsburgs

The European royal dynasties of the Early Modern Age provide a useful framework for human inbreeding research. In this article, consanguineous marriage, inbreeding depression and the purging of deleterious alleles within a consanguineous population are investigated in the Habsburgs, a royal dynasty with a long history of consanguinity over generations. Genealogical information from a number of historical sources was used to compute kinship and inbreeding coefficients for the Habsburgs. Marriages contracted by the Habsburgs from 1450 to 1750 presented an extremely high mean kinship (0.0628 {plus minus} 0.009), which was the result of the matrimonial policy conducted by the dynasty to establish political alliances through marriage. A strong inbreeding depression for both infant and child survival was detected in the progeny of 71 Habsburg marriages in the period 1450-1800. The inbreeding load for child survival experienced a pronounced decrease from 3.98 {plus minus} 0.87 in the period 14...

早期近代欧洲王室王朝为人类近亲繁殖研究提供了实用框架。本文以哈布斯堡王朝——一个世代具有长期近亲婚配历史的王室家族——为对象，探讨了近亲婚姻（consanguineous marriage）、近亲繁殖衰退（inbreeding depression）及近亲群体内有害等位基因（deleterious alleles）的清除现象。研究利用多份历史来源的谱系信息，计算了哈布斯堡家族成员的亲缘系数（kinship coefficient）与近交系数（inbreeding coefficient）。1450至1750年间哈布斯堡家族的婚姻呈现极高平均亲缘系数（0.0628±0.009），这是该王朝通过婚姻建立政治联盟的婚姻政策（matrimonial policy）所致。1450-1800年间哈布斯堡家族71桩婚姻的后代中，检测到婴儿与儿童存活率存在显著的近亲繁殖衰退。儿童存活率的近交负荷（inbreeding load）从14...时期的3.98±0.87显著下降...