Molecular weight (DA)
105kD
Immunogen
Synthesized peptide derived from human LONM AA range: 398-448
Specificity
This antibody detects endogenous levels of LONM at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.251% sodium azide.
Dilution rate
WB 1:500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
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. [provided by RefSeq, Feb 2013],
Function
function:Required for intramitochondrial proteolysis. Catalyzes the initial steps of protein degradation. Hydrolyzes ATP and degrades protein and peptide substrates in an ATP-dependent manner. Binds to single-stranded but not to double-stranded DNA.,similarity:Belongs to the peptidase S16 family.,similarity:Contains 1 Lon domain.,tissue specificity:Duodenum, heart, lung and liver, but not thymus.,
Other name
Lon protease homolog, mitochondrial (EC 3.4.21.-) (LONHs) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)
Human protein sequence Database
P36776
Mouse protein sequence database
Q8CGK3
Rat protein sequence database
Q924S5
Cellular localization
Mitochondrion matrix .
Tissue expression
Duodenum, heart, lung and liver, but not thymus.