Complement component 1s: Difference between revisions

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'''Complement component 1s''' (C1s) is a protein enzyme that plays a crucial role in the [[complement system]], which is an essential part of the [[innate immune system]]. The complement system helps to clear pathogens from an organism. C1s is part of the C1 complex, which also includes [[Complement component 1q|C1q]] and [[Complement component 1r|C1r]], and is involved in the classical pathway of complement activation.
{{DISPLAYTITLE:Complement Component 1}}


== Structure and Function ==
== Complement Component 1 ==
C1s is a serine protease that, once activated, is responsible for cleaving and activating the next components in the pathway, [[Complement component 4|C4]] and [[Complement component 2|C2]], to form the C3 convertase, C4b2a. This enzyme complex then cleaves [[Complement component 3|C3]], marking pathogens for destruction and initiating a series of immune responses including opsonization, inflammation, and cell lysis.
[[File:Droga_klasyczna.png|thumb|right|Diagram of the classical pathway of complement activation, showing the role of Complement Component 1.]]


The activation of C1s is tightly regulated. It is initially part of the C1 complex, which is bound to C1q. The activation occurs when C1q binds to the Fc region of [[Immunoglobulin G|IgG]] or [[Immunoglobulin M|IgM]] that is bound to antigens on the surface of a pathogen. This binding causes a conformational change in C1q, which activates C1r. C1r then cleaves and activates C1s.
Complement Component 1 (C1) is a crucial protein complex in the [[complement system]], which is part of the [[innate immune system]]. The complement system enhances the ability of [[antibodies]] and [[phagocytic cells]] to clear microbes and damaged cells from an organism, promotes [[inflammation]], and attacks the pathogen's cell membrane.


== Genetic and Molecular Aspects ==
=== Structure ===
The gene encoding C1s is located on chromosome 12 in humans. Mutations in this gene can lead to deficiencies in the complement system, which can result in increased susceptibility to infections and autoimmune diseases. The molecular weight of the C1s protein is approximately 85 kDa.
C1 is a complex of three different proteins: C1q, C1r, and C1s. These proteins are organized in a specific structure that allows C1 to function effectively in the immune response.


== Clinical Significance ==
* '''C1q''' is a large molecule composed of 18 polypeptide chains, forming a structure that can bind to the [[Fc region]] of [[immunoglobulin]]s.
Deficiencies in C1s are rare but can lead to a range of immune disorders. For example, a deficiency in C1s can result in a form of [[Systemic lupus erythematosus|systemic lupus erythematosus (SLE)]], an autoimmune disease where the body's immune system attacks its own tissues. Additionally, because the complement system plays a role in inflammation, dysregulation of C1s activity can contribute to inflammatory diseases.
* '''C1r''' and '''C1s''' are serine proteases that are activated in a cascade manner.


Inhibitors of C1s are being explored as potential therapeutic agents for diseases involving excessive complement activation, such as hereditary angioedema, a condition characterized by sudden and severe swelling of the skin and mucous membranes.
=== Function ===
C1 is the first component of the [[classical pathway]] of complement activation. It is responsible for recognizing and binding to the [[antigen-antibody complex]]. Upon binding, C1 undergoes a conformational change that activates C1r, which in turn activates C1s. Activated C1s then cleaves and activates the next components in the cascade, C4 and C2, leading to the formation of the [[C3 convertase]].


== Research Directions ==
=== Activation ===
Research on C1s is focused on understanding its structure-function relationships, regulation of its activity, and its role in disease. Studies on C1s inhibitors for therapeutic purposes are also a significant area of interest, particularly for conditions where the complement system's activation contributes to disease pathology.
The activation of C1 is tightly regulated to prevent excessive inflammation and tissue damage. C1 is activated when C1q binds to the Fc region of IgG or IgM antibodies that are bound to antigens. This binding triggers the activation of C1r and C1s, initiating the complement cascade.


== See Also ==
=== Role in Disease ===
Deficiencies or dysfunctions in C1 can lead to immune system disorders. For example, C1 deficiency can result in increased susceptibility to infections and autoimmune diseases such as [[systemic lupus erythematosus]].
 
== Related Pages ==
* [[Complement system]]
* [[Complement system]]
* [[Innate immune system]]
* [[Classical pathway]]
* [[Serine protease]]
* [[Antibody]]
* [[Hereditary angioedema]]
* [[Immune system]]
 
== References ==
<references/>


[[Category:Immune system]]
[[Category:Immunology]]
[[Category:Complement system]]
[[Category:Complement system]]
{{medicine-stub}}

Latest revision as of 05:33, 16 February 2025


Complement Component 1[edit]

Diagram of the classical pathway of complement activation, showing the role of Complement Component 1.

Complement Component 1 (C1) is a crucial protein complex in the complement system, which is part of the innate immune system. The complement system enhances the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promotes inflammation, and attacks the pathogen's cell membrane.

Structure[edit]

C1 is a complex of three different proteins: C1q, C1r, and C1s. These proteins are organized in a specific structure that allows C1 to function effectively in the immune response.

  • C1q is a large molecule composed of 18 polypeptide chains, forming a structure that can bind to the Fc region of immunoglobulins.
  • C1r and C1s are serine proteases that are activated in a cascade manner.

Function[edit]

C1 is the first component of the classical pathway of complement activation. It is responsible for recognizing and binding to the antigen-antibody complex. Upon binding, C1 undergoes a conformational change that activates C1r, which in turn activates C1s. Activated C1s then cleaves and activates the next components in the cascade, C4 and C2, leading to the formation of the C3 convertase.

Activation[edit]

The activation of C1 is tightly regulated to prevent excessive inflammation and tissue damage. C1 is activated when C1q binds to the Fc region of IgG or IgM antibodies that are bound to antigens. This binding triggers the activation of C1r and C1s, initiating the complement cascade.

Role in Disease[edit]

Deficiencies or dysfunctions in C1 can lead to immune system disorders. For example, C1 deficiency can result in increased susceptibility to infections and autoimmune diseases such as systemic lupus erythematosus.

Related Pages[edit]