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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. Alternatively spliced transcript variants have been found for this gene.
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Protein Aliases: h LonHS; hLON ATP-dependent protease; Lon protease homolog, mitochondrial; Lon protease-like protein; LONHs; LONP; LONP1; Mitochondrial ATP-dependent protease Lon; mitochondrial lon protease-like protein; Serine protease 15
Gene Aliases: CODASS; hLON; LON; LonHS; LONP; LONP1; PIM1; PRSS15
UniProt ID: (Human) P36776
Entrez Gene ID: (Human) 9361