SPATA18
Description
The SPATA18 (spermatogenesis associated 18) is a protein-coding gene located on chromosome 4.
SPATA18, also known as MIEAP, plays a crucial role in maintaining mitochondrial quality by mediating the repair or degradation of damaged mitochondria. It acts as a mediator of mitochondrial protein catabolic process (MALM) by facilitating the accumulation of lysosomal hydrolases within the mitochondrial matrix, leading to the degradation of damaged proteins. Additionally, SPATA18 promotes the formation of vacuole-like structures (MIV) that engulf and degrade unhealthy mitochondria by accumulating lysosomes. The interaction of SPATA18 with BNIP3 and BNIP3L/NIX at the mitochondrial outer membrane regulates the opening of a pore in the mitochondrial double membrane, enabling the translocation of lysosomal proteins from the cytoplasm to the mitochondrial matrix.
SPATA18 is also known as Mieap, SPETEX1.