The only nuclear gene responsible for recurrent myoglobinuria in mitochondrial myopathy is LPIN1. LPIN1 is a phosphatidic acid phosphohydrolase, and its impairment causes a phospholipid composition defect leading to the accumulation of lysophospholipids in the inner mitochondrial membrane, which act as detergents, thus promoting muscle breakdown [8]. Here, LPIN1 is linked to Mitochondrial myopathy.