Anti-LONP1 Rabbit pAb
- 100ug (NP23263) Please inquiry
Antibody type:Primary antibody
Label:Unconjugated
Modification:Unmodification
Isotype:Rabbit IgG
Host:Rabbit
Application:WB
Purify method:Affinity purified
Species:Human,Mouse,Rat
Gene Name:LONP1
Synonyms:LON; LONP; PIM1; hLON; LonHS; CODASS; PRSS15
Gene Synonyms:
Gene Full Name:lon peptidase 1, mitochondrial
Gene Infomation: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]
Antigen:Recombinant fusion protein containing a sequence corresponding to amino acids 120-400 of human LONP1 (NP_004784.2).
Antigen Synonyms:Lon protease homolog, mitochondrial
Clonality:Polyclonal antibody
Source:Human
Reaction:
Form:Liquid
Tested Applications:
- Western blot (1:100 to 1:500)
- Immunofluorescence (1:50 to 1:400)
- Immunohistochemistry (1:200 to 1:500)
- Flow cytometry analysis (1:200 to 1:500)
- Enzyme-linked Immunosorbent Assay (1:100-1:5000)
Clone:
Dilution:WB1:500 -1:2000(Optimal dilutions should be determined by the end user)
Mole Mass:105kDa
Location:Mitochondrion
Concentration:
Sequence Similarity:
Gene Id:
SwissProt ID:P36776
Unigene:9361
Nucleotide Accession:
Tissue specificity:Duodenum, heart, lung and liver, but not thymus.Gene expression databases
Storage:Store at -20°C. Avoid freeze / thaw cycles.
Buffer condition:PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Background:
Molar 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 (PubMed:12198491, PubMed:15870080, PubMed:17420247, PubMed:8248235). 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 (PubMed:17579211, PubMed:28377575).
Western Blot:Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.