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Protein / target

ATP-dependent Clp protease proteolytic subunit, mitochondrial

Encoded byCLPPQ16740Homo sapiensSwiss-Prot
UniProt: Q16740Ensembl: ENSG00000125656HGNC: HGNC:2084NCBI Gene: 8192
Small-molecule tractable
Druggability
Structure with Ligand
1
Research papers

Protein at a glance

Biological role

Serine-type endopeptidase

Strongest disease association

Genetic Diseases, Inborn

Via encoding gene CLPP · Genetic evidence · score 0.31

Research activity

Emerging research

1 papers · latest 2024

Derived from structured UniProt, Open Targets and literature data on this page.

Protein profile

UniProt 2026_02

Canonical identity and biological annotation from UniProt.

Function overview

Protease component of the ClpXP complex that cleaves peptides and various proteins in an ATP-dependent process.

View complete UniProt function annotation

Protease component of the ClpXP complex that cleaves peptides and various proteins in an ATP-dependent process. Has low peptidase activity in the absence of CLPX. The ClpXP 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 (PubMed:11923310, PubMed:15522782). Cleaves PINK1 in the mitochondrion (PubMed:22354088)

Subcellular location

Mitochondrion matrix
Domains and Gene Ontology detail (14)

Gene Ontology

  • Cendopeptidase Clp complex
  • Cmitochondrial matrix
  • Cmitochondrion
  • FATP-dependent peptidase activity
  • FATPase binding
  • Fendopeptidase activity
  • Fidentical protein binding
  • Fpeptidase activity
  • Fserine-type endopeptidase activity
  • Pmembrane protein proteolysis
  • Pmitochondrial protein catabolic process
  • Pprotein quality control for misfolded or incompletely synthesized proteins

277 aa · 30 kDa

Biological roles

What this protein does, drawn together from its UniProt function, Gene Ontology terms and Reactome pathways.

ProteolysisUniProt · GO
View supporting evidence

Proteolysis

  • ·Protease component of the ClpXP complex that cleaves peptides and various proteins in an…
  • ·ATP-dependent peptidase activity
  • ·endopeptidase activity
  • ·peptidase activity

Concepts derived from UniProt GO Reactome — each badge above shows which sources supported that role.

Translational evidence

Open Targets 26

Why this target matters therapeutically, strongest evidence first. Disease associations are gene-level (via the gene that encodes this protein) and open into the full confidence synthesis; the development universe, tractability and safety annotations are target-level, from Open Targets.

Strongest disease associations · via encoding gene CLPP

Gene-level evidence surfaced through the gene CLPP that encodes this protein — not a direct protein–disease relationship. Ranked by Forefront's causal-directness weighting, so genetically- and clinically-evidenced diseases lead over ones that merely share the literature.

Genetic Diseases, Inborn
0.32Limited support

Genetic evidence dominant · Open Targets 0.19

Neoplasms
0.12Preliminary

Literature evidence dominant · Open Targets 0.09 · no direct causal or clinical evidence

Parkinson's Disease
0.11Preliminary

Literature evidence dominant · Open Targets 0.09 · no direct causal or clinical evidence

Carcinoma, Hepatocellular
0.10Preliminary

Literature evidence dominant · Open Targets 0.08 · no direct causal or clinical evidence

View evidence synthesis (4)
Genetic Diseases, InbornLimited support
0.32
agreement 0.18–0.46
Genetic99%Literature1%

Open Targets aggregate 0.19 · 2 independent evidence families

NeoplasmsPreliminary
0.12
agreement 0.00–0.39
Literature100%

Open Targets aggregate 0.09 · 1 independent evidence family · no direct causal or clinical evidence

Parkinson's DiseasePreliminary
0.11
agreement 0.00–0.38
Literature100%

Open Targets aggregate 0.09 · 1 independent evidence family · no direct causal or clinical evidence

Carcinoma, HepatocellularPreliminary
0.10
agreement 0.00–0.37
Literature100%

Open Targets aggregate 0.08 · 1 independent evidence family · no direct causal or clinical evidence

The evidence agreement range shows how closely the independent evidence families agree — it is not a statistical confidence interval, and nothing here is fitted to outcome data. Derived from Open Targets evidence types under Forefront weighting; the per-type scores above show the calculation.

Show all associations
Genetic Diseases, Inborn0.19
Neoplasms0.09
Parkinson's Disease0.09
Carcinoma, Hepatocellular0.08

Tractability

Small molecules — Emerging

Feasibility evidence (structure with ligand) — no clinical-stage drug of this modality recorded.

Protein degraders — Emerging

Feasibility evidence (database ubiquitination and half-life data) — no clinical-stage drug of this modality recorded.

View underlying tractability evidence (4)
SM · Structure with LigandPR · Database UbiquitinationPR · Half-life DataPR · Small Molecule Binder

Raw Open Targets tractability assessment buckets, by modality.

Research activity

1 papers · to 2024

Papers linked directly to this protein. This is the protein's own literature — descriptor-derived papers are kept separate below.

Most cited

CLPB Deficiency Associated Neonatal Cavitating Leukoencephalopathy: A Potential Pathomechanism Underlying Neurologic Disorder.2 cites

Darouich S · Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society · 2024

Recent

CLPB Deficiency Associated Neonatal Cavitating Leukoencephalopathy: A Potential Pathomechanism Underlying Neurologic Disorder.

Darouich S · Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society · 2024

Europe PMC papers linked directly to this protein.