Recombinant Human Lon protease homolog, mitochondrial (LONP1), Truncated

Uniprot Id

P36776

Target Species

Human

Target Name

LONP1

Target Full Name

Lon protease homolog, mitochondrial

Target Function

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, helicase Twinkle (TWNK) and the large ribosomal subunit protein bL32m. bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not.

Target Involvement

CODAS syndrome (CODASS)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Peptidase S16 family

Target Tissue Specificity

Duodenum, heart, lung and liver, but not thymus.

Target Research Area

Cancer

Target Synonyms

hLON; hLON ATP dependent protease; LON; lon peptidase 1, mitochondrial; LON protease; Lon protease homolog; Lon protease like protein; Lon protease-like protein; LONHs; LONM_HUMAN; LONP; Lonp1; MGC1498; mitochondrial; Mitochondrial ATP dependent protease Lon; Mitochondrial ATP-dependent protease Lon; Mitochondrial lon peptidase 1; PIM1; Protease serine 15; PRSS15; Serine protease 15

Target Background

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.