C1q Human, Liquid

Complement Component C1q Human, Liquid
Cat. No.
BT13898
Source
Human Plasma.
Synonyms
Component C1q, Complement C1q, Complement Component C1q, C1q.
Appearance

Sterile Filtered solution.

Purity

Greater than 95.0% as determined by SDS-PAGE.

Usage
Prospec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
Shipped with Ice Packs
In Stock

Description

Human Complement Component C1q produced in Human plasma having a molecular mass of 410 kDa.

Product Specs

Introduction
C1q, the first component of the classical complement pathway, forms the C1 complex with C1r and C1s. It activates upon binding to immune complexes, triggering the complement cascade. As a collectin family glycoprotein (410-462 kDa), C1q comprises six globular heads connected to collagen-like tails. These heads bind specifically to IgG's CH2 or IgM's CH3 domain, requiring attachment to at least two heavy chains for activation. This ensures activation only when bound to multivalent antigens. C1q's primary function is clearing immune complexes and apoptotic bodies, crucial for preventing autoimmunity. Deficiencies in C1q or other classical pathway components can lead to SLE. C1q binds to apoptotic cells like keratinocytes, endothelial cells, and lymphocytes, aiding their removal alongside C3. This clearance of autoantigens helps avoid immune system stimulation. However, prolonged exposure to C1q bound to immune complexes or apoptotic bodies can trigger autoimmunity against C1q, disrupting complement function. Additionally, C1q deficiency may hinder the elimination of autoreactive B cells. Along with other recognition proteins, C1q binds to lupus antigens (dsDNA, nuclear proteins), activating the complement system. Anti-C1q antibodies, present in autoimmune and infectious diseases like GN and SLE, hold clinical significance due to their negative predictive value.
Description

Human Complement Component C1q, derived from human plasma, has a molecular weight of 410 kDa.

Physical Appearance

Sterile Filtered solution.

Formulation

The solution contains 10 mM HEPES and 300mM NaCl, with a pH of 7.2.

Stability

Human C1q can be stored at 4°C for 2-4 weeks if the entire vial will be used. For longer storage, freeze below -20°C. Adding a carrier protein (0.1% HSA or BSA) is recommended for long-term storage. Avoid repeated freezing and thawing.

Purity

The purity is greater than 95.0% as determined by SDS-PAGE analysis.

Human Virus Test

Plasma from each donor undergoes testing to ensure it's negative for antibodies against HIV-1, HIV-2, HCV, and HBSAG.

Synonyms
Component C1q, Complement C1q, Complement Component C1q, C1q.
Source
Human Plasma.

Product Science Overview

Structure and Composition

Complement Component C1q is a glycoprotein composed of 18 polypeptide chains. These chains are organized into three nonidentical subunits, known as A, B, and C, with molecular weights of 29 kDa, 26 kDa, and 19 kDa, respectively . The structure of C1q is quite unique, resembling a bouquet of flowers, with six A-chains, six B-chains, and six C-chains forming a complex structure . Each chain contains a collagen-like region near the N-terminus and a C-terminal globular region .

Function and Mechanism

C1q is the first component of the classical pathway of the complement system. It is present in normal human serum at a concentration of approximately 70 µg/ml . C1q, along with C1r and C1s, forms the C1 complex. This complex is responsible for the activation of the classical complement pathway, which is triggered by the binding of C1q to immune complexes containing immunoglobulin G (IgG) or immunoglobulin M (IgM) .

Upon binding to these immune complexes, C1q undergoes a conformational change that activates C1r and C1s, converting them into proteolytic enzymes. These enzymes then cleave other complement proteins, leading to a cascade of reactions that result in the opsonization of pathogens, recruitment of inflammatory cells, and lysis of target cells .

Clinical Relevance

C1q plays a vital role in the clearance of apoptotic cells and immune complexes, preventing the accumulation of cellular debris and autoantigens that could trigger autoimmune responses . Deficiencies in C1q are associated with a higher risk of developing autoimmune diseases such as systemic lupus erythematosus . Additionally, C1q has been found to bind to apoptotic bodies of human keratinocytes, vascular endothelial cells, and lymphocytes, facilitating their clearance .

Applications in Research and Medicine

Complement Component C1q is widely used in research to study the mechanisms of the complement system and its role in immune responses. It is also used in diagnostic assays to evaluate the functionality of the classical complement pathway. In therapeutic contexts, understanding the role of C1q can aid in the development of treatments for autoimmune diseases and conditions involving immune complex-mediated pathology .

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