Dynasty's downfall ... it was all in the breeding

Charles II of Spain

The Hapsburg dynasty was one of the most important and influential royal families in Europe dating back more than 500 years and producing rulers in Austria, Hungary, Belgium, the Netherlands and the German empire. Then, in 1700, it suffered a sudden demise of its Spanish branch. Now scientists believe they have come up with a definitive explanation.

A study of the extended family tree of the House of Hapsburg has found that the last Spanish Hapsburg King, Charles II, was the offspring of a marriage that was almost as genetically inbred as an incestuous relationship between a brother and sister or parent and child.

Scientists have found the Hapsburg fashion of marrying their relatives to keep their dynastic heritage intact had dire consequences for subsequent generations, which culminated in the last heir to the Spanish throne being sickly and impotent.

Charles II of Spain was nicknamed El Hechizado - The Hexed - because people thought that his disabilities were the result of sorcery. Now a study has found that he was so inbred that he probably suffered from at least two inherited disorders.

Despite his deformities and severe health problems, Charles had married twice in the hope of continuing the rule of the Hapsburgs, but he was incapable of fathering an heir and died childless at the age of 39.

Scientists believe they can show just how inbred Charles was following a study of more than 3000 relatives of the Hapsburg family extending over 16 generations. The researchers found that his "inbreeding coefficient" - a measure of the proportion of inbred genes he had inherited from his parents - was on a par with that of the offspring of an incestuous marriage.

Professor Gonzalo Alvarez, of the University of Santiago de Compostela in Spain, found the Hapsburgs suffered a far higher child mortality than the general population, even though the family did not experience the poverty-related health problems faced by many people at the time.

They also suffered a higher incidence of physical deformities, best exemplified by the famous "Hapsburg lip", a disfiguringly prominent lower jaw caused by an inherited medical condition called mandibular prognathism, when the lower jaw grows faster than the upper jaw.

Charles II not only suffered an extreme version of the Hapsburg lip, his tongue was said to be so big for his mouth that he had difficulty speaking and drooled. He also suffered from an oversized head, intestinal upsets, convulsions and, according to his first wife, premature ejaculation.

"He was unable to speak until the age of four, and could not walk until the age of eight. He was short, weak and quite lean and thin. He was described as a person showing very little interest in his surroundings," Professor Alvarez said.

The medical problems of Charles II were the direct result of many generations of interbreeding between relatives within the extended Hapsburg dynasty, according to the study published in the online journal Plos One.

Charles' father, Philip IV, was the uncle of his mother, Mariana of Austria; his great-grandfather, Philip II, was also the uncle of his great-grandmother, Anna of Austria; and his grandmother, Maria Anna of Austria, was simultaneously his aunt.

There were many marriages between first and third cousins within the Hapsburg family, as well as between uncles and nieces.

This meant that down the generations the degree of genetic inbreeding gradually built up.

The medical dangers of such a high level of inbreeding is that dangerously defective genes, which are usually recessive, can come together in one individual and so manifest themselves as an ailment. This is why the offspring of first-cousin marriages are at higher risk of inherited disorders.

Professor Alvarez suggested that Charles II had inherited genes that caused two genetic disorders. One was a hormone imbalance called pituitary hormone deficiency, which would have affected his growth and development, and the other was a kidney problem that led to a metabolic disorder which caused impotence and infertility.

"His muscular weakness at a young age, rickets, haematuria [blood in the urine] and big head relative to his body size could be attributed to this genetic disorder," he said.

"In this way, we may speculate that most of the symptoms showed by Charles II could be explained by two genetic disorders."

CHARLES II OF SPAIN

* Charles II of Spain was nicknamed El Hechizado - The Hexed - because it was thought that his physical and mental disabilities were the result of sorcery.

* He had an enormous misshapen head, his jaw was so long that his two rows of teeth could not meet meaning he was unable to chew and his tongue was so large that he found it difficult to speak and drooled.

* He was small and sickly looking and lacked the strength to walk confidently.

* Despite being married twice, he failed to father any children.

* Scientists believe his poor health and deformities were the direct result of many generations of breeding between close relatives in the extended Hapsburg dynasty.

* His father, Philip IV, was the uncle of his mother, Mariana of Austria; his great-grandfather, Philip II, was the uncle of his great-grandmother, Anna of Austria; his grandmother, Maria Anna of Austria, was also his aunt.

* When Charles II died in 1700 at the age of 39, the line of the Spanish Hapsburgs died with him.

THE HAPSBURGS

* The House of Hapsburg supplied all of the formally elected Holy Roman Emperors between 1452 and 1740, as well as rulers of Spain and the Austrian Empire.

* Originally from Switzerland, the dynasty first reigned in Austria, which they ruled for more than six centuries. But a series of dynastic marriages brought Burgundy, Spain, Bohemia, Hungary and other territories into the inheritance.

* To keep power within the Hapsburg family, there were many marriages between first and third cousins, as well as unions between uncles and nieces and more remote family members.

QUOTES

Ferdinand I:
* "I am emperor - I will have noodles."
* "To govern is easy, to sign one's name is difficult."

- INDEPENDENT