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LONP1 encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. Additionally we are shipping Lon Peptidase 1, Mitochondrial Antibodies (82) and many more products for this protein.
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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 (PMID:18378094). Alternatively spliced transcript variants have been found for this gene.
lon peptidase 1, mitochondrial
, protease, serine, 15
, lon protease homolog, mitochondrial-like
, hLON ATP-dependent protease
, lon protease homolog, mitochondrial
, mitochondrial ATP-dependent protease Lon
, mitochondrial lon protease-like protein
, serine protease 15
, lon protease-like protein
, ATP-dependent Lon protease
, hypothetical protein