Predicted to enable ATP-dependent peptidase activity and single-stranded DNA binding activity. Involved in protein catabolic process. Predicted to be located in mitochondrial matrix. Expressed in head and tail. Human ortholog(s) of this gene implicated in CODAS syndrome. Is an ortholog of human LONP1 (lon peptidase 1, mitochondrial).
Lon protease (Lon) encodes a conserved ATP-stimulated serine protease. It is encoded in the nucleus and targeted to the mitochondrial matrix, where it contributes to mitochondrial protein turnover.
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]
Involved in several processes, including negative regulation of BMP signaling pathway; negative regulation of DNA endoreduplication; and positive regulation of growth rate. Located in adherens junction and plasma membrane. Expressed in head; hypodermis; intestinal cell; intestine; and tail.
Enables growth factor binding activity. Involved in several processes, including negative regulation of transmembrane receptor protein serine/threonine kinase signaling pathway; regulation of cell migration; and regulation of mesodermal cell fate specification. Located in cell surface. Expressed in hypodermis and intestine.
Involved in nematode male tail tip morphogenesis and regulation of growth. Predicted to be located in extracellular space. Expressed in hyp4; hyp7 syncytium; and intestine.
Predicted to be a structural constituent of collagen and cuticulin-based cuticle. Involved in body morphogenesis and regulation of growth. Located in annuli extracellular matrix. Expressed in several structures, including H1L.apa; H1L.appa; H1L.p; cuticle; and hypodermal cell. Human ortholog(s) of this gene implicated in congenital myasthenic syndrome 5. Is an ortholog of human COLQ (collagen like tail subunit of asymmetric acetylcholinesterase).
In human, peroxisomes function primarily to catalyze fatty acid beta-oxidation and, as a by-product, produce hydrogen peroxide and superoxide. The protein encoded by this gene is an ATP-dependent protease that likely plays a role in maintaining overall peroxisome homeostasis as well as proteolytically degrading peroxisomal proteins damaged by oxidation. The protein has an N-terminal Lon N substrate recognition domain, an ATPase domain, a proteolytic domain, and, in some isoforms, a C-terminal peroxisome targeting sequence. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jan 2017]
Predicted to be involved in regulation of signal transduction. Predicted to be located in collagen-containing extracellular matrix and plasma membrane. Is an ortholog of O. volvulus Ovo-lon-2.