Property Summary

NCBI Gene PubMed Count 17
Grant Count 87
R01 Count 40
Funding $14,655,347.23
PubMed Score 459.26
PubTator Score 107.92

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (3)

Disease log2 FC p
Multiple myeloma 1.184 0.004
psoriasis 1.100 0.001
ovarian cancer 1.300 0.000

Synonym

Accession Q16740 B2R4W5
Symbols DFNB81
PRLTS3

Gene

PDB

1TG6  

Pathway (1)

Gene RIF (8)

PMID Text
26606371 ClpP proteases from E. coli, S. aureus, and human mitochondria exhibit preferences for certain amino acids in the P1, P2, and P3 positions .
25083874 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.
23541340 Perrault syndrome is caused by recessive mutations in CLPP.
23360988 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.
20877624 Observational study of gene-disease association. (HuGE Navigator)
18378094 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
16115876 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
15522782 the N-terminal peptide of ClpP is a structural component of the substrate translocation channel and may play an important functional role as well

AA Sequence

MWPGILVGGARVASCRYPALGPRLAAHFPAQRPPQRTLQNGLALQRCLHATATRALPLIPIVVEQTGRGE      1 - 70
RAYDIYSRLLRERIVCVMGPIDDSVASLVIAQLLFLQSESNKKPIHMYINSPGGVVTAGLAIYDTMQYIL     71 - 140
NPICTWCVGQAASMGSLLLAAGTPGMRHSLPNSRIMIHQPSGGARGQATDIAIQAEEIMKLKKQLYNIYA    141 - 210
KHTKQSLQVIESAMERDRYMSPMEAQEFGILDKVLVHPPQDGEDEPTLVQKEPVEAAPAAEPVPAST       211 - 277
//

Text Mined References (23)

PMID Year Title
26606371 2016 Barrel-shaped ClpP Proteases Display Attenuated Cleavage Specificities.
25956234 2015 Exome analysis identified a novel missense mutation in the CLPP gene in a consanguineous Saudi family expanding the clinical spectrum of Perrault Syndrome type-3.
25944712 2015 N-terminome analysis of the human mitochondrial proteome.
25083874 2014 Stochastic but highly coordinated protein unfolding and translocation by the ClpXP proteolytic machine.
24275569 2014 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
23541340 2013 Perrault syndrome is caused by recessive mutations in CLPP, encoding a mitochondrial ATP-dependent chambered protease.
23360988 2013 Human CLPP reverts the longevity phenotype of a fungal ClpP deletion strain.
21269460 2011 Initial characterization of the human central proteome.
20877624 2010 Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression.
19892738 2009 Global profiling of protease cleavage sites by chemoselective labeling of protein N-termini.
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