Ankyrin repeat and LEM domain-containing protein 1 (ANKLE1)

Ankyrin repeat and LEM domain-containing protein 1 (ANKLE1) is a mammalian endonuclease characterized by a C-terminal GIY-YIG motif and an internal LEM domain. It cleaves branched DNA species, such as four-way junctions and Y-structures, and shuttles between the nucleus and cytoplasm. In normal physiology, ANKLE1 is primarily expressed in erythroblast lineage cells, where it facilitates mitochondrial genome degradation during erythropoiesis. Ectopic or high expression in non-hematopoietic cells leads to mitochondrial and genome instability, triggers DNA damage responses, and is associated with increased risk for cancer (notably breast and ovarian). Mechanistically, ANKLE1’s activity can induce mitophagy, metabolic reprogramming to increased glycolysis, and may confer resistance to apoptosis. While essential for mitochondrial clearance in maturing red blood cells, its broader physiological and pathological roles outside the erythroid lineage remain incompletely defined[1][2][3][4].

Other names

ANKRD41LEM3FLJ39369LEMD6ankyrin repeat domain-containing protein 41LEM-domain containing protein 3LEM domain containing 6