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Mouse Anti-LONP1 Recombinant Antibody (3B2) (CBMAB-A7101-LY)

The product is antibody recognizes PRSS15. The antibody 3B2 immunoassay techniques such as: WB, ELISA.
See all LONP1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
3B2
Antibody Isotype
IgG2a, κ
Application
WB, ELISA

Basic Information

Immunogen
PRSS15 (NP_004784.2, 661 a.a. ~ 761 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Specificity
Human
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
LONP1
Introduction
This gene encodes a mitochondrial matrix protein in the Lon family of ATP-dependent proteases. A similar E. coli protein regulates gene expression by targeting specific regulatory proteins for degradation. This protein binds a specific sequence in the light and heavy chain promoters of the mitochondrial genome which are involved in regulation of DNA replication and transcription. [provided by RefSeq]
Entrez Gene ID
9361
UniProt ID
P36776
Alternative Names
LON; LONP; LonHS; MGC1498; PIM1; PRSS15; hLON
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).
Biological Process
Cellular response to oxidative stressManual Assertion Based On ExperimentIDA:UniProtKB
Chaperone-mediated protein complex assemblyManual Assertion Based On ExperimentIBA:GO_Central
Mitochondrial DNA metabolic process1 PublicationNAS:UniProtKB
Mitochondrial genome maintenance1 PublicationNAS:UniProtKB
Mitochondrion organizationManual Assertion Based On ExperimentIMP:UniProtKB
Oxidation-dependent protein catabolic processManual Assertion Based On ExperimentIMP:UniProtKB
Protein quality control for misfolded or incompletely synthesized proteinsManual Assertion Based On ExperimentIBA:GO_Central
Proteolysis involved in cellular protein catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Response to hypoxiaManual Assertion Based On ExperimentIEP:UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
CODAS syndrome (CODASS):
A rare syndrome characterized by the combination of cerebral, ocular, dental, auricular, and skeletal features. These include developmental delay, craniofacial anomalies, cataracts, ptosis, median nasal groove, delayed tooth eruption, hearing loss, short stature, delayed epiphyseal ossification, metaphyseal hip dysplasia, and vertebral coronal clefts.

Xu, Z., Fu, T., Guo, Q., Zhou, D., Sun, W., Zhou, Z., ... & Gan, Z. (2022). Disuse-associated loss of the protease LONP1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength. Nature Communications, 13(1), 894.

Sheng, X., Liu, C., Yan, G., Li, G., Liu, J., Yang, Y., ... & Ding, L. (2022). The mitochondrial protease LONP1 maintains oocyte development and survival by suppressing nuclear translocation of AIFM1 in mammals. EBioMedicine, 75.

Zhao, K., Huang, X., Zhao, W., Lu, B., & Yang, Z. (2022). LONP1-mediated mitochondrial quality control safeguards metabolic shifts in heart development. Development, 149(6), dev200458.

Shin, C. S., Meng, S., Garbis, S. D., Moradian, A., Taylor, R. W., Sweredoski, M. J., ... & Chan, D. C. (2021). LONP1 and mtHSP70 cooperate to promote mitochondrial protein folding. Nature Communications, 12(1), 265.

Maneix, L., Sweeney, M. A., Lee, S., Iakova, P., Moree, S. E., Sahin, E., ... & Catic, A. (2021). The mitochondrial protease LonP1 promotes proteasome inhibitor resistance in multiple myeloma. Cancers, 13(4), 843.

Pollecker, K., Sylvester, M., & Voos, W. (2021). Proteomic analysis demonstrates the role of the quality control protease LONP1 in mitochondrial protein aggregation. Journal of Biological Chemistry, 297(4).

Gibellini, L., De Gaetano, A., Mandrioli, M., Van Tongeren, E., Bortolotti, C. A., Cossarizza, A., & Pinti, M. (2020). The biology of Lonp1: More than a mitochondrial protease. International Review of Cell and Molecular Biology, 354, 1-61.

Besse, A., Brezavar, D., Hanson, J., Larson, A., & Bonnen, P. E. (2020). LONP1 de novo dominant mutation causes mitochondrial encephalopathy with loss of LONP1 chaperone activity and excessive LONP1 proteolytic activity. Mitochondrion, 51, 68-78.

Venkatesh, S., Li, M., Saito, T., Tong, M., Rashed, E., Mareedu, S., ... & Suzuki, C. K. (2019). Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo. Journal of molecular and cellular cardiology, 128, 38-50.

Ghosh, J. C., Seo, J. H., Agarwal, E., Wang, Y., Kossenkov, A. V., Tang, H. Y., ... & Altieri, D. C. (2019). Akt phosphorylation of mitochondrial Lonp1 protease enables oxidative metabolism and advanced tumor traits. Oncogene, 38(43), 6926-6939.

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For research use only. Not intended for any clinical use.

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