| Function | 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. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, DELE1, helicase Twinkle (TWNK) and the large ribosomal subunit protein MRPL32/bL32m. MRPL32/bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not. 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. |
| Protein Name | Lon Protease Homolog - MitochondrialLonhsLon Protease-Like ProteinLonpMitochondrial Atp-Dependent Protease LonSerine Protease 15 |
| Database Links | Reactome: R-HSA-9837999Reactome: R-HSA-9841251 |
| Cellular Localisation | Mitochondrion Matrix |
| Alternative Protein Names | Lon Protease Homolog - Mitochondrial proteinLonhs proteinLon Protease-Like Protein proteinLonp proteinMitochondrial Atp-Dependent Protease Lon proteinSerine Protease 15 proteinLONP1 proteinPRSS15 protein |
Information sourced from Uniprot.org
