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C1 esterase inhibitor [human] liquid

Shire · Phase 3 active Small molecule

C1 esterase inhibitor replaces or augments the deficient or dysfunctional C1 inhibitor protein, which normally suppresses excessive activation of the contact system and complement cascade.

C1 esterase inhibitor replaces or augments the deficient or dysfunctional C1 inhibitor protein, which normally suppresses excessive activation of the contact system and complement cascade. Used for Hereditary angioedema (HAE) Types I and II — acute attack treatment and prophylaxis, Acquired angioedema (AAE) — acute attack treatment.

At a glance

Generic nameC1 esterase inhibitor [human] liquid
SponsorShire
Drug classSerine protease inhibitor; plasma-derived protein replacement
TargetC1 esterase inhibitor (C1-INH); Factor XIIa, Factor XIa, Kallikrein
ModalitySmall molecule
Therapeutic areaImmunology; Rare Genetic Disorders
PhasePhase 3

Mechanism of action

C1 esterase inhibitor (C1-INH) is a serine protease inhibitor that regulates the initial steps of the intrinsic coagulation pathway, fibrinolytic system, and complement cascade. In hereditary angioedema (HAE) and acquired angioedema (AAE), deficiency or dysfunction of C1-INH leads to uncontrolled activation of these cascades, resulting in excessive bradykinin production and angioedema. Replacement with human C1-INH restores normal regulation and prevents or treats acute angioedema attacks.

Approved indications

Common side effects

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