LONP1 protein, also known as Lon protease homolog 1, is a nuclear-encoded mitochondrial AAA+ serine protease that plays a crucial role in maintaining mitochondrial homeostasis. It is highly conserved across species and is composed of three functional domains: an N-terminal domain for substrate recognition, an AAA+ domain for ATP binding and hydrolysis, and a protease domain for protein degradation. LONP1 forms a hexameric complex in the mitochondrial matrix and is responsible for degrading misfolded, oxidized, and damaged proteins to prevent their accumulation and maintain mitochondrial proteostasis. Additionally, LONP1 has chaperone-like functions in protein folding and assembly, and it regulates mitochondrial DNA (mtDNA) replication and transcription by interacting with mitochondrial transcription factor A (TFAM). Its dysfunction is associated with various diseases, including mitochondrial disorders and cancer, making it a potential target for therapeutic interventions.
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