Property Summary

NCBI Gene PubMed Count 17
PubMed Score 459.26
PubTator Score 107.92

Knowledge Summary


No data available


  Disease Sources (5)

Disease Target Count
Gonadal dysgenesis XX type deafness 4
Disease Target Count P-value
ovarian cancer 8492 1.8626038180584E-4
psoriasis 6685 0.00102820134604989
Multiple myeloma 1328 0.00435948788115006
Disease Target Count Z-score Confidence
Perrault syndrome 12 6.567 3.3
Disease Target Count
Perrault syndrome 3 1


  Differential Expression (3)

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


Accession Q16740 B2R4W5
Symbols DFNB81




  Ortholog (13)

Species Source
Chimp OMA EggNOG
Macaque OMA EggNOG
Mouse OMA EggNOG Inparanoid
Rat OMA EggNOG Inparanoid
Dog OMA EggNOG Inparanoid
Horse OMA EggNOG Inparanoid
Cow OMA EggNOG Inparanoid
Platypus EggNOG Inparanoid
Anole lizard OMA EggNOG Inparanoid
Xenopus OMA EggNOG Inparanoid
Zebrafish OMA EggNOG Inparanoid
C. elegans OMA EggNOG Inparanoid
Fruitfly EggNOG Inparanoid

Pathway (1)

Gene RIF (8)

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


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.