Aconitase deficiency is characterized by myopathy with severe exercise intolerance and deficiencies of skeletal muscle succinate dehydrogenase and aconitase. The disease is MONDO_0009706 (hereditary myopathy with lactic acidosis due to ISCU deficiency). Also known as: ISCU myopathy, aconitase deficiency, iron-sulfur cluster deficiency myopathy, myopathy with exercise intolerance, Swedish type.