The LONP1 Polyclonal Antibody from MyBioSource.com is a Rabbit Polyclonal antibody to lon, LONP1, PIM, PIM1, and PIM2. This antibody recognizes Human, Mouse, and Rat antigen. The LONP1 Polyclonal Antibody has been shown to work in the following applications: Immunofluorescence, Immunohistochemistry, Immunoprecipitation, and Western Blot.
Description
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