CLPP

Protein identityUniProt · HPA

UniProt accession

Q16740

Gene

CLPP

Ensembl

ENSG00000125656

Chromosome

19

Canonical length

277 aa

Protein class

Disease related genes, Enzymes, Human disease related genes, Potential drug targets, Predicted intracellular proteins

Subcellular location

Mitochondria

OverviewNCBI Gene

The protein encoded by this gene belongs to the peptidase family S14 and hydrolyzes proteins into small peptides in the presence of ATP and magnesium. The protein is transported into mitochondrial matrix and is associated with the inner mitochondrial membrane. [provided by RefSeq, Jul 2008]

Canonical amino-acid sequenceUniProt

277 residues, UniProt reviewed canonical sequence.

>Q16740|CLPP
     1  MWPGILVGGA RVASCRYPAL GPRLAAHFPA QRPPQRTLQN GLALQRCLHA TATRALPLIP
    61  IVVEQTGRGE RAYDIYSRLL RERIVCVMGP IDDSVASLVI AQLLFLQSES NKKPIHMYIN
   121  SPGGVVTAGL AIYDTMQYIL NPICTWCVGQ AASMGSLLLA AGTPGMRHSL PNSRIMIHQP
   181  SGGARGQATD IAIQAEEIMK LKKQLYNIYA KHTKQSLQVI ESAMERDRYM SPMEAQEFGI
   241  LDKVLVHPPQ DGEDEPTLVQ KEPVEAAPAA EPVPAST

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against CLPP can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.41

Highest tissue expression

153 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about CLPP.

Disease | AllUniProt

Conditions CLPP is implicated in, by any mechanism.

• Perrault syndrome 3 (PRLTS3) MIM:614129

Disease | GeneticClinVar

23 pathogenic / likely-pathogenic of 241 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Perrault syndrome 3
• Perrault syndrome
• Autosomal recessive hearing impairment with normal menstrual cycles
• Perrault syndrome 1

Disease | ImmuneIEDB

Conditions an epitope on CLPP was assayed in.

• skin melanoma T cell

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.56

gnomAD pLI

0.57

gnomAD missense Z

1.95

DepMap mean gene effect

-0.15

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• membrane protein proteolysis
• mitochondrial protein catabolic process
• protein quality control for misfolded or incompletely synthesized proteins
• proteolysis
• proteolysis involved in protein catabolic process

Molecular functions

• ATP-dependent peptidase activity
• ATPase binding
• endopeptidase activity
• identical protein binding
• peptidase activity
• serine-type endopeptidase activity

Cellular components

• endopeptidase Clp complex
• mitochondrial matrix
• mitochondrion

Protein domainsUniProt · Pfam · InterPro

• ClpP/crotonase-like domain superfamily
• ATP-dependent Clp protease proteolytic subunit
• ClpP, Ser active site
• Clp protease proteolytic subunit /Translocation-enhancing protein TepA
• ClpP, histidine active site
• Clp protease

KeywordsUniProt

• Acetylation
• Deafness
• Hydrolase
• Mitochondrion
• Protease
• Serine protease
• Transit peptide

InteractionsUniProt · HPA

Protein binding partners of CLPP in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• SNCA
• UQCRFS1