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ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein.
Gene Name: | lon peptidase 1, mitochondrial |
Family/Subfamily: | Protease , Serine S16 |
Synonyms: | LONP1, HLON ATP-dependent protease, HLON, LON, Lon peptidase 1, mitochondrial, Lon protease, LONP, Mitochondrial lon peptidase 1, Protease, serine, 15, PRSS15, Lon protease-like protein, LonHS, Serine protease 15, Protease Serine 15 |
Target Sequences: | NM_004793 NP_004784.2 P36776 |
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