免疫原
Synthesized peptide derived from human LONM AA range: 398-448
特异性
This antibody detects endogenous levels of LONM at Human/Mouse/Rat
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.251% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（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],
功能
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.,
基因名称（Gene Name）
LONP1 PRSS15
其他名称
Lon protease homolog, mitochondrial (EC 3.4.21.-) (LONHs) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)
细胞定位
Mitochondrion matrix .
组织表达
Duodenum, heart, lung and liver, but not thymus.
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