Polygamy and genetics

There’s a new twist in the case of the FLDS, and the children taken from the YFZ ranch. As described in this Time article, the insular and endogamous community of the FLDS has become plagued with a very rare genetic disorder: fumarase deficiency. This autosomal recessive disorder caused by a mutation in the gene for the enzyme that converts fumarate to malate in the mitochondria, making this one of the few known deficiencies of the citric acid cycle. The result is brain malformation, deformity, grand mal seizures, severe mental retardation (IQ near 25), and encephalopathy.
(Thus we see not only the dangers of inbreeding, but also the founder effect in action).

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