Protein / target
Plasma protease C1 inhibitor
Protein at a glance
Biological role
Serine-type endopeptidase inhibitor
Strongest disease association
Angioedema
Therapeutic position
Established drug target
Research activity
Emerging research
Derived from structured UniProt, Open Targets and literature data on this page.
Protein profile
Canonical identity and biological annotation from UniProt.
Function overview
Serine protease inhibitor, which acrs as a regulator of the classical complement pathway.
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Serine protease inhibitor, which acrs as a regulator of the classical complement pathway (PubMed:10946292, PubMed:11527969, PubMed:3458172, PubMed:6416294). Forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases (PubMed:10946292, PubMed:3458172, PubMed:6416294). May also regulate blood coagulation, fibrinolysis and the generation of kinins (PubMed:8495195). Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein (PubMed:8495195)
Subcellular location
Domains and Gene Ontology detail (12)Hide
Gene Ontology
- Cblood microparticle
- Cendoplasmic reticulum lumen
- Cextracellular exosome
- Cextracellular matrix
- Cextracellular region
- Cextracellular space
- Cplatelet alpha granule lumen
- Fserine-type endopeptidase inhibitor activity
- Pblood circulation
- Pblood coagulation
- Pfibrinolysis
- Pnegative regulation of complement activation, lectin pathway
Biological roles
What this protein does, drawn together from its UniProt function, Gene Ontology terms and Reactome pathways.
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Haemostasis
- ·Serine protease inhibitor, which acrs as a regulator of the classical complement pathway…
- ·platelet alpha granule lumen
- ·blood coagulation
- ·fibrinolysis
Concepts derived from UniProt GO Reactome — each badge above shows which sources supported that role.
Translational evidence
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 SERPING1
Gene-level evidence surfaced through the gene SERPING1 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.
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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.
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Drug development
1 compounds recorded · 1 approved
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Open Targets known-drugs universe. Drug name and highest clinical stage only — the disease relationship is NOT read from this slice (it carries trial-context noise); approved indications come from the canonical graph above.
Tractability
Antibodies — Emerging
Protein degraders — Emerging
Other modalities — Strong
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Raw Open Targets tractability assessment buckets, by modality.
Research activity
Papers linked directly to this protein. This is the protein's own literature — descriptor-derived papers are kept separate below.
Most cited
Recent
Europe PMC papers linked directly to this protein.