This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013],
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Function:
Function:Required for intramitochondrial proteolysis. Catalyzes the initial steps of protein degradation. Hydrolyzes ATP and degrades protein and peptide substrates in an ATP-dependent manner. Binds to single-stranded but not to double-stranded DNA.,similarity:Belongs to the peptidase S16 family.,similarity:Contains 1 Lon domain.,tissue specificity:Duodenum, heart, lung and liver, but not thymus.,