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By Mayo Clinic staffThe cause of familial Mediterranean fever is a gene defect on chromosome 16 (each person normally has 46 chromosomes, in 23 pairs). The gene, called MEFV, carries the genetic code for a protein called pyrin, which is involved in regulating inflammation. More than 50 different mutations in the MEFV gene have been identified — including one (M694V) that's found more frequently in Arabs and North African Jews. Although it's not certain exactly how this defect causes familial Mediterranean fever, it may be that people with this mutation don't make pyrin, hence, inflammatory responses aren't regulated and may be quite severe.
Familial Mediterranean fever is inherited as an autosomal recessive trait, meaning that you must inherit the mutated gene from each parent in order to develop the condition.