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Article

C1 inhibitor serpin domain structure reveals the likely mechanism of heparin potentiation and conformational disease.

The Journal of biological chemistry - 20 Jul 2007

Beinrohr László, Harmat Veronika, Dobó József, Lörincz Zsolt, Gál Péter, Závodszky Péter

Abstract excerpt

C1 inhibitor, a member of the serpin family, is a major down-regulator of inflammatory processes in blood. Genetic deficiency of C1 inhibitor results in hereditary angioedema, a dominantly inheritable, potentially lethal disease. Here we report the first crystal structure of the serpin domain of human C1 inhibitor, representing a previously unreported latent form, which explains functional consequences of several...

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PMID: 17488724
DOI: 10.1074/jbc.M700841200

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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C1 inhibitor serpin domain structure reveals the likely mechanism of heparin potentiation and conformational disease.PMID 17488724Read ↗

Intermittent C1-Inhibitor Deficiency Associated with Recessive Inheritance: Functional and Structural Insight.Semantic proximity Read ↗

Structural and functional aspects of C1-inhibitor.Semantic proximity Read ↗

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COOH-terminal substitutions in the serpin C1 inhibitor that cause loop overinsertion and subsequent multimerization.Semantic proximity Read ↗

Restriction of C1-inhibitor activity in hereditary angioedema by dominant-negative effects of disease-associated SERPING1 gene variantsSemantic proximity Read ↗

The functional integrity of the serpin domain of C1-inhibitor depends on the unique N-terminal domain, as revealed by a pathological mutant.Semantic proximity Read ↗

Dominant-negative SERPING1 variants cause intracellular retention of C1 inhibitor in hereditary angioedemaSemantic proximity Read ↗

Pathophysiology of Hereditary AngioedemaSemantic proximity Read ↗

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Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment.Semantic proximity Read ↗

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