LONP1 Gene

lon peptidase 1, mitochondrial

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]

LONP1 Gene Set

From Pathway Commons Protein-Protein Interactions

interacting proteins for LONP1 from the Pathway Commons Protein-Protein Interactions dataset.

lonp1dependent Gene Set

From GeneRIF Biological Term Annotations

genes co-occuring with the biological term lonp1dependent in literature-supported statements describing functions of genes from the GeneRIF Biological Term Annotations dataset.