ID:LONM_HUMAN DESCRIPTION: RecName: Full=Lon protease homolog, mitochondrial; EC=3.4.21.-; AltName: Full=LONHs; AltName: Full=Lon protease-like protein; Short=LONP; AltName: Full=Mitochondrial ATP-dependent protease Lon; AltName: Full=Serine protease 15; Flags: Precursor; 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. 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. SUBUNIT: Homohexamer or homoheptamer. Organized in a ring with a central cavity. DNA and RNA binding is stimulated by substrate and inhibited by ATP binding. Interacts with PEO1 and mitochondrial DNA polymerase subunit POLG. SUBCELLULAR LOCATION: Mitochondrion matrix. TISSUE SPECIFICITY: Duodenum, heart, lung and liver, but not thymus. SIMILARITY: Belongs to the peptidase S16 family. SIMILARITY: Contains 1 Lon domain. SEQUENCE CAUTION: Sequence=CAA52291.1; Type=Erroneous initiation;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P36776
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0000002 mitochondrial genome maintenance GO:0001666 response to hypoxia GO:0006508 proteolysis GO:0006515 misfolded or incompletely synthesized protein catabolic process GO:0007005 mitochondrion organization GO:0007568 aging GO:0009725 response to hormone GO:0010044 response to aluminum ion GO:0030163 protein catabolic process GO:0032042 mitochondrial DNA metabolic process GO:0034599 cellular response to oxidative stress GO:0034622 cellular macromolecular complex assembly GO:0051260 protein homooligomerization GO:0051603 proteolysis involved in cellular protein catabolic process GO:0070407 oxidation-dependent protein catabolic process
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Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
