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LONP1 encodes a mitochondrial matrix protein in the Lon family of ATP-dependent proteases.
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Human Polyclonal LONP1 Primary Antibody pour WB - ABIN1881504
Merra, Lago, Ricci, Antuzzi, Gasbarrini, Gasbarrini, Ghirlanda: Splenomegaly as a primary manifestation of Gaucher disease in a young adult woman. dans Case reports in gastroenterology 2011
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Human Polyclonal LONP1 Primary Antibody pour ICC, IF - ABIN4331301
Gozal, Duong, Gearing, Cheng, Hanfelt, Funderburk, Peng, Lah, Levey: Proteomics analysis reveals novel components in the detergent-insoluble subproteome in Alzheimer's disease. dans Journal of proteome research 2009
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mitochondrial ATP-dependent Lon protease may serve as a potential biomarker for cancer diagnosis and novel target for the development of anticancer drugs and for predicting of the efficiency and effectiveness of chemotherapy of a variety of cancers.
We demonstrate that Lon plays a key role in glioma cell hypoxic survival and mitochondrial respiration, and propose Lon as a promising therapeutic target in the treatment of malignant gliomas.
Some features were not consistent with CODAS syndrome but overlapped with Marinesco-Sjogren syndrome, a multisystem disorder caused by a mutation in SIL1 (Montrer SIL1 Anticorps). An atypical mutation site may result in atypical presentation of the LONP1 mutation
LONP1 function and implication in human aging and disease was reviewed.
we observed that Lon protease downregulation is linked to a higher lipofuscinogenesis whereas the application of the mitochondrial-targeted antioxidant mitoTEMPO is able to prevent the accumulation of this protein aggregate.
Lon preferentially degrades the phosphorylated subunits of CcO (Montrer RYR1 Anticorps) and plays a role in the regulation of CcO (Montrer RYR1 Anticorps) activity in hypoxia and ischemia/reperfusion injury.
Lon protease (Lonp1), which is a key inductive of mitochondrial unfolded protein response (UPR(mt)) and is required to maintain the mitochondrial quality, was greatly induced in H. pylori infected gastric epithelial cells.
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.
these findings suggest that Lon protease may be particularly vulnerable to inactivation in conditions associated with elevated oxidative stress resulting in mitochondrial dysfunction and neuron cell death, an early event in the pathogenesis of Parkinson's disease
LONP1 knockdown inhibits cellular proliferation and tumor and metastasis formation, whereas its overexpression increases tumorigenesis.
Epidermal growth factor (Montrer EGF Anticorps) up-regulates the transcription of ATP-dependent protease through extracellular signal-regulated protein kinase (Montrer CDK7 Anticorps)- and 1-phosphatidylinositol 3-kinase (Montrer PIK3CG Anticorps)-dependent pathways.
Modulation of Lon protease activity during aging and oxidative stress.
The correlative effect of Lon and ClpP (Montrer CLPP Anticorps) 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 (Montrer CLPP Anticorps) proteases in FRDA (Montrer FXN Anticorps).
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