Function
LonP1 is an ATP-dependent serine protease localized to the mitochondrial matrix. Its primary function is the selective degradation of misfolded, unassembled, or oxidatively damaged polypeptides, as well as certain short-lived regulatory proteins. LonP1 may also exhibit chaperone activity in the assembly of inner mitochondrial membrane protein complexes. Furthermore, it plays a role in regulating mitochondrial gene expression and maintaining the integrity of the mitochondrial genome through site-specific binding to mitochondrial DNA. Evidence suggests LonP1 is involved in the degradation of the transcription factor, ATFS-1, within the mitochondrion.