CLPP Antibody

說明書

中文名稱：

CLPP兔多克隆抗體
貨號：

CSB-PA005577GA01HU
規格：

￥3,900
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q16740
基因名：

CLPP
別名：

ATP dependent protease ClpAP (E coli) proteolytic subunit antibody; ATP dependent protease ClpAP proteolytic subunit antibody; ATP dependent protease ClpAP proteolytic subunit human antibody; ATP dependent proteolytic subunit homolog (E coli) antibody; ATP dependent proteolytic subunit homolog antibody; Caseinolytic peptidase ATP dependent proteolytic subunit homolog antibody; Caseinolytic protease ATP dependent proteolytic subunit E coli antibody; clpP antibody; ClpP caseinolytic peptidase ATP dependent proteolytic subunit antibody; ClpP caseinolytic peptidase ATP dependent proteolytic subunit homolog antibody; CLPP_HUMAN antibody; Endopeptidase Clp antibody; mitochondrial antibody; Putative ATP dependent Clp protease proteolytic subunit mitochondrial antibody; Putative ATP dependent Clp protease proteolytic subunit antibody; Putative ATP-dependent Clp protease proteolytic subunit antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human CLPP
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC,IF
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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：
1. Autophagy compensates impaired energy metabolism in CLPXP-deficient Podospora anserina strains and extends healthspan. PMID: 28449241
2. 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
3. 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
4. Data suggest that tumors exploit ClpXP-directed proteostasis to maintain mitochondrial bioenergetics, buffer oxidative stress, and enable metastatic competence. PMID: 27389535
5. Data indicate endopeptidase Clp (ClpP) mutation identified in two patients with Perrault syndrome type 3 in a Turkish family. PMID: 27087618
6. 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
7. 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
8. 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
9. Perrault syndrome is caused by recessive mutations in CLPP. PMID: 23541340
10. 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
11. 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
12. 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.
數據庫鏈接：

HGNC: 2084

OMIM: 601119

KEGG: hsa:8192

STRING: 9606.ENSP00000245816

UniGene: Hs.515092

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