Anti-LONP1/Lon Antibody Picoband® Fluoro647 Conjugated

Lon protease homolog, mitochondrial is an enzyme that in humans is encoded by the LONP1 gene. It is mapped to 19p13.3. 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.

Lon protease homolog, mitochondrial; LONHs; Lon protease-like protein; LONP; Mitochondrial ATP-dependent protease Lon; Serine protease 15; LONP1; 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, 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.

1. Petukhova GV, Grigorenko VG, Lykov IP, Yarovoi SV, Lipkin VM, Gorbalenya AE (February 1994) . Cloning and sequence analysis of cDNA for a human homolog of eubacterial ATP-dependent Lon proteases. FEBS Letters. 340 (1–2) : 25–8. 2. Pinti M, Gibellini L, Liu Y, Xu S, Lu B, Cossarizza A (December 2015) . Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer. Cellular and Molecular Life Sciences. 72 (24) : 4807–24.