Human lon peptidase 1, mitochondrial ELISA Kit | LONP1 elisa kit
Human Lon protease homolog, mitochondrial, LONP1 ELISA Kit
NCBI and Uniprot Product Information
NCBI 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. [provided by RefSeq, Feb 2013]
Uniprot Description
PRSS15: 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. Belongs to the peptidase S16 family.
Protein type: RNA-binding; Protease; DNA-binding; Mitochondrial; EC 3.4.21.-
Chromosomal Location of Human Ortholog: 19p13.2
Cellular Component: membrane; mitochondrion; mitochondrial matrix; cytoplasm
Molecular Function: G-quadruplex DNA binding; protein binding; single-stranded RNA binding; sequence-specific DNA binding; serine-type endopeptidase activity; ATP-dependent peptidase activity; ADP binding; ATP binding; single-stranded DNA binding
Biological Process: mitochondrion organization and biogenesis; mitochondrial genome maintenance; proteolysis involved in cellular protein catabolic process; misfolded or incompletely synthesized protein catabolic process; response to hormone stimulus; response to hypoxia; chaperone-mediated protein complex assembly; mitochondrial DNA metabolic process; protein homooligomerization; response to aluminum ion; aging
Disease: Codas Syndrome
Research Articles on LONP1
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Product Notes
The Human LONP1 lonp1 (Catalog #AAA9340799) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9340799 ELISA Kit recognizes Human LONP1. It is sometimes possible for the material contained within the vial of "lon peptidase 1, mitochondrial, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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