LONP1 Antibody – Rabbit Polyclonal

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]

PRSS15: ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single- stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site- specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein. Belongs to the peptidase S16 family.

Protein type: RNA-binding; DNA-binding; Mitochondrial; EC 3.4.21.-; Protease

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: mitochondrion; membrane; mitochondrial matrix; cytoplasm

Molecular Function: G-quadruplex DNA binding; protein binding; single-stranded RNA binding; sequence-specific DNA binding; ATP-dependent peptidase activity; serine-type endopeptidase activity; ADP binding; ATP binding; single-stranded DNA binding

Biological Process: mitochondrion organization and biogenesis; mitochondrial genome maintenance; proteolysis involved in cellular protein catabolic process; misfolded or incompletely synthesized protein catabolic process; response to hypoxia; response to hormone stimulus; chaperone-mediated protein complex assembly; mitochondrial DNA metabolic process; protein homooligomerization; response to aluminum ion; aging

Disease: Codas Syndrome