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LONP1 encodes a mitochondrial matrix protein in the Lon family of ATP-dependent proteases. Additionally we are shipping Lon Peptidase 1, Mitochondrial Proteins (4) and many more products for this protein.
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Human Polyclonal LONP1 Primary Antibody for WB - ABIN1881504
Merra, Lago, Ricci, Antuzzi, Gasbarrini, Gasbarrini, Ghirlanda: Splenomegaly as a primary manifestation of Gaucher disease in a young adult woman. in Case reports in gastroenterology 2011
Show all 5 references for ABIN1881504
Mutations of Lon, which likely impair its chaperone properties, are at the basis of a genetic inherited disease named the cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome. (Review)
Inhibition of Lon protease by triterpenoids alters mitochondria and is associated to cell death in human cancer cells.
Lon downregulation attenuated hypoxia-induced cardiomyocyte apoptosis through a reduction of reactive oxygen species level.
LONP1 encodes an enzyme of bacterial ancestry that participates in protein turnover within the mitochondrial matrix, and mutations in its ATP-binding and proteolytic domains cause CODAS syndrome.
A review on the recent discoveries concerning Lon Protease functions. [review]
These results suggest that the mechanism underlying cell survival regulated by Lon is mediated by the maintenance of the protein stability of Hsp60 (show HSPD1 Antibodies)-mtHsp70 (show HSPA9 Antibodies) complex.
Silencing of SIRT3 (show SIRT3 Antibodies) increased the levels of Lon protein and of its acetylation, suggesting that Lon is a target of SIRT3 (show SIRT3 Antibodies), likely at K917.
We establish a link between LONP1 and CODAS syndrome in humans.
Lonp1 has a protective role against ochratoxin a induced cytotoxicity in kidney cells.
StAR proteolysis is executed by at least 2 mitochondrial proteases, the matrix LON protease and the inner membrane complexes of the metalloproteases AFG3L2 (show AFG3L2 Antibodies) and AFG3L2 (show AFG3L2 Antibodies):SPG7/paraplegin (show SPG7 Antibodies).
LONP1 knockdown inhibits cellular proliferation and tumor and metastasis formation, whereas its overexpression increases tumorigenesis.
Epidermal growth factor (show EGF Antibodies) up-regulates the transcription of ATP-dependent protease through extracellular signal-regulated protein kinase (show CDK7 Antibodies)- and 1-phosphatidylinositol 3-kinase (show PIK3CG Antibodies)-dependent pathways.
Modulation of Lon protease activity during aging and oxidative stress.
The correlative effect of Lon and ClpP (show CLPP Antibodies) upregulation on loss of mitochondrial Fe-S proteins during the progression of the disease may suggest that Fe-S proteins are potential targets of Lon and ClpP (show CLPP Antibodies) proteases in FRDA (show FXN Antibodies).
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.
lon peptidase 1, mitochondrial
, protease, serine, 15
, lon protease homolog, mitochondrial-like
, hLON ATP-dependent protease
, lon protease homolog, mitochondrial
, mitochondrial ATP-dependent protease Lon
, mitochondrial lon protease-like protein
, serine protease 15
, lon protease-like protein
, ATP-dependent Lon protease
, hypothetical protein