Lon protease homolog, mitochondrial is an enzyme that in humans is encoded by the LONP1gene. It is mapped to 19p13.3. 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.
Optimal dilution of the LONP1 antibody should be determined by the researcher.
Amino acids R137-A320 from the human protein were used as the immunogen for the LONP1 antibody.
Store the LONP1 antibody at -20oC.
