Target intelligence / Profile preview

Complement component 1 complex (C1)

Target
C1
Molecular classification
Enzyme, Recognition molecule, Protein complex, Scaffold protein
01

Overview

The **Complement component 1 complex (C1)** is a multiprotein assembly initiating the classical complement pathway, comprising C1q (recognition molecule), and two serine proteases C1r and C1s, forming the C1qC1r2C1s2 structure[1][2][5]. Activation occurs when C1q binds antigen–antibody complexes or other ligands, leading to proteolytic activation of C1r and then C1s. Activated C1s cleaves complement proteins C4 and C2, driving downstream immune responses and inflammation. Beyond complement activation, C1 complex is involved in cell debris clearance, modulation of immune and cellular functions, and interacts with diverse molecules including cell surface receptors and apoptotic cells, illustrating its role as a versatile node in immunity and cellular biology[2][3][4]. Pathological dysregulation of C1 or its subunits is linked to a range of diseases including autoimmunity and chronic inflammatory conditions, making C1 a critical biomarker and therapeutic target[4].

Other names
Complement C1C1 complexC1qC1r2C1s2C1qr2s2C1qC1rC1s
02

Mechanism of action

Inhibition of C1 activation (blockade of subunit interactions, often targeting the protease activity of C1r or C1s, or the recognition by C1q); Prevention of complement cascade initiation

03

Biological functions

Immune response (classical complement pathway activation)Pathogen recognitionClearance of cell debris and apoptotic cellsRegulation of cellular eventsSignal transduction (involved in Wnt signaling through interactions with Frizzled receptors)Induction of inflammation
04

Disease associations

InflammationInfection (bacterial, viral, etc.)Autoimmune disease (dysregulation or deficiency implicated in lupus and other autoimmune disorders)Neurodegenerative disease (roles in aging CNS and possibly Alzheimer's disease)Cardiovascular disease (roles in atherosclerosis)Cancer (potential role in tumor growth regulation)
05

Safety considerations

Inhibition may increase infection risk (by reducing innate immune defense)Potential for immune complex disease if complement activation is insufficientOff-target effects on tissue repair and inflammatory regulation
06

Interacting drugs

C1 inhibitors (e.g. C1 esterase inhibitor [C1-INH]; drugs for hereditary angioedema)

1 more in the full profile.

07

Biomarkers

Serum levels of C1q, C1r, C1s (for complement system activation)Functional assays of complement activity (CH50, etc.)C1-INH activity (in hereditary angioedema)Detection of C1q in tissue for autoimmune diagnosis

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