DDHD domain-containing phospholipase A1; mitochondrial matrix enzyme with sn-1-specific activity, hydrolyzing cardiolipin, PE, PC, PG and PA; implicated in remodeling of mitochondrial phospholipids; antagonistically regulated by Aft1p and Aft2p; in humans, mutations in DDHD1 and DDHD2 genes cause specific types of hereditary spastic paraplegia, while DDL1-defective yeast share similar phenotypes such as mitochondrial dysfunction and defects in lipid metabolism
