Anti-LONP1 antibody

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Product name Anti-LONP1 antibody
Short Description Rabbit polyclonal against LONP1
Description 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.
Applications WB
Dilution range WB 1:500 - 1:2000
Protein Name Lon Protease Homolog - Mitochondrial
Lonhs
Lon Protease-Like Protein
Lonp
Mitochondrial Atp-Dependent Protease Lon
Serine Protease 15
Immunogen Recombinant fusion protein containing a sequence corresponding to amino acids 120-400 of human LONP1 (NP_004784. 2).
Storage Instruction Store at -20°C. Avoid freeze / thaw cycles.
Note For Research Use Only (RUO).
Host Rabbit
Clonality Polyclonal
Reactivity Human, Mouse, Rat
Conjugation Unconjugated
Purification Affinity purification
Isotype IgG
Formulation PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Gene ID 9361
Gene Symbol LONP1
Molecular Weight Calculated: 85kDa/100kDa/106kDa. Observed: 106kDa
Alternative Names Anti-Lon Protease Homolog - Mitochondrial antibody
Anti-Lonhs antibody
Anti-Lon Protease-Like Protein antibody
Anti-Lonp antibody
Anti-Mitochondrial Atp-Dependent Protease Lon antibody
Anti-Serine Protease 15 antibody
Anti-LONP1 PRSS15 antibody
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.
Cellular Localization Mitochondrion Matrix
Tissue Specificity Duodenum, heart, lung and liver, but not thymus.
Swiss-Prot Key LONM_HUMAN
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