IHC image of CSB-PA618094LA01HU diluted at 1:500 and staining in paraffin-embedded human prostate tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
IHC image of CSB-PA618094LA01HU diluted at 1:500 and staining in paraffin-embedded human prostate cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
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貨期:
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用途:
For Research Use Only. Not for use in diagnostic or therapeutic procedures.
Protease component of the Clp complex that cleaves peptides and various proteins in an ATP-dependent process. Has low peptidase activity in the absence of CLPX. The Clp complex can degrade CSN1S1, CSN2 and CSN3, as well as synthetic peptides (in vitro) and may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates. Cleaves PINK1 in the mitochondrion.
基因功能參考文獻:
Autophagy compensates impaired energy metabolism in CLPXP-deficient Podospora anserina strains and extends healthspan. PMID: 28449241
we demonstrate that a strong mitochondrial cardiomyopathy and diminished respiration due to DARS2 deficiency can be alleviated by the loss of CLPP, leading to an increased de novo synthesis of individual OXPHOS subunits. PMID: 27154400
ere we present eight families affected by Perrault syndrome. In five families we identified novel or previously reported variants in HSD17B4, LARS2, CLPP and C10orf2 PMID: 26970254
Data suggest that tumors exploit ClpXP-directed proteostasis to maintain mitochondrial bioenergetics, buffer oxidative stress, and enable metastatic competence. PMID: 27389535
Data indicate endopeptidase Clp (ClpP) mutation identified in two patients with Perrault syndrome type 3 in a Turkish family. PMID: 27087618
ClpP proteases from E. coli, S. aureus, and human mitochondria exhibit preferences for certain amino acids in the P1, P2, and P3 positions . PMID: 26606371
Optical trapping to assay single-molecule ClpXP unfolding and translocation of substrates consisting of domains with varying stabilities and sequences; find that ClpXP unfolds most domains by a single pathway, with kinetics that depend on the native fold and structural stability. PMID: 25083874
Deletion of PaClpP, leads to an unexpected healthy phenotype and increased lifespan of the fungal ageing model organism Podospora anserina and This phenotype can be reverted by expression of human ClpP. PMID: 23360988
Perrault syndrome is caused by recessive mutations in CLPP. PMID: 23541340
the N-terminal peptide of ClpP is a structural component of the substrate translocation channel and may play an important functional role as well PMID: 15522782
hClpX can regulate the appearance of hClpP peptidase activity in mitochondria and might affect the nature of the degradation products released during ATP-dependent proteolytic cycles PMID: 16115876
We propose that decreased levels of mitochondrial proteases Lon and ClpP may allow heat shock protein 60 substrate proteins to go through more folding attempts PMID: 18378094
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相關疾病:
Perrault syndrome 3 (PRLTS3)
亞細胞定位:
Mitochondrion matrix.
蛋白家族:
Peptidase S14 family
組織特異性:
Detected in liver (at protein level). Predominantly expressed in skeletal muscle. Intermediate levels in heart, liver and pancreas. Low in brain, placenta, lung and kidney.