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LONP1
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]
Protein class
Disease related genes, Enzymes, Human disease related genes, Potential drug targets
Predicted location
Intracellular
Single cell type specificity
Cell type enhanced (Theca cells)
Immune cell specificity
Low immune cell specificity
Cell line specificity
Low cell line specificity
Interaction
Homohexamer (PubMed:14739292, 25369343, 20222013). Organized in a ring with a central cavity (PubMed:25369343, 20222013). The ATP-binding and proteolytic domains (AP-domain) form a hexameric chamber, while the N-terminal domain is arranged as a trimer of dimers (PubMed:27632940). DNA and RNA binding is stimulated by substrate and inhibited by ATP binding. Interacts with TWNK and mitochondrial DNA polymerase subunit POLG (PubMed:14739292).
Molecular function
DNA-binding, Hydrolase, Protease, Serine protease
More Types Infomation
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- Species Reactivity: Human
- Application: ELISA, WB
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For Research Use Only. Not For Clinical Use.