C4 Human

Complement C4 Human
Cat. No.
BT16410
Source

Human Plasma.

Synonyms

Complement C4-A, Acidic complement C4, C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2, C4A, CO4, CPAMD2.

Appearance

Sterile filtered solution.

Purity

Greater than 90.0% as determined by SDS-PAGE.

Usage
THE BioTek'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 C4 produced in Human plasma having a molecular mass of 205 kDa.

Product Specs

Introduction

C4, a key component of the complement system, is a protein found in human plasma. This protein exists as three disulfide-linked chains and lacks glycosylation. C4 is crucial for activating both the lectin and classical complement pathways. These pathways, upon initiation, form enzyme complexes that attach to target surfaces and cleave C4. This cleavage generates C4a, an anaphylatoxin, and activates C4b. C4b, similar to C3, possesses a highly reactive thioester that can covalently bind to amino or hydroxyl groups on target surfaces. Humans commonly express two variants of C4, C4A and C4B, while most animals with a single C4 type typically express C4B.

Description

Human Complement C4, with a molecular weight of 205 kDa, derived from human plasma.

Physical Appearance

A clear, sterile solution that has passed through a filter.

Formulation

This solution of C4 protein is buffered with 10mM sodium phosphate and 145mM NaCl, maintaining a pH of 7.3.

Stability

For optimal stability, keep Human C4 refrigerated at 4°C. If using within 2-4 weeks, the entire vial can be stored at this temperature. For longer storage, freeze below -20°C. Consider adding a carrier protein like 0.1% HSA or BSA for extended storage. To maintain product integrity, avoid repeated freezing and thawing.

Purity

Purity level exceeding 90.0% as determined by SDS-PAGE analysis.

Human Virus Test

Each plasma donation undergoes rigorous testing to ensure the absence of antibodies against HIV-1, HIV-2, HCV, HTLV-I & II. Additionally, it is tested for STS and HBSAG, with negative results for all.

Synonyms

Complement C4-A, Acidic complement C4, C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2, C4A, CO4, CPAMD2.

Source

Human Plasma.

Product Science Overview

Origin and Structure

Complement C4 originates from the human leukocyte antigen (HLA) system. It is encoded by two genes, C4A and C4B, located on chromosome 6. These genes are part of the RCCX gene complex, which includes other genes such as CYP21. The C4 protein exists in two forms: C4A and C4B, which differ slightly in their structure and function .

Function in the Immune System

C4 is involved in the classical and lectin pathways of the complement system. These pathways are activated when antibodies bind to antigens, forming immune complexes. C4 is cleaved into two fragments, C4a and C4b, upon activation. C4b binds to the surface of pathogens, marking them for destruction by other immune cells. This process is known as opsonization. C4a, on the other hand, acts as an anaphylatoxin, which promotes inflammation by attracting immune cells to the site of infection .

Genetic Variability and Disease Association

C4 is one of the most polymorphic proteins in the complement system, meaning it has many genetic variations. These variations can influence the levels of C4A and C4B proteins in individuals, affecting their immune response. Deficiencies or dysfunctions in C4 can lead to increased susceptibility to infections and autoimmune diseases. For example, individuals with low levels of C4 are more prone to conditions such as systemic lupus erythematosus (SLE) and schizophrenia .

Historical Context

The study of C4 began with the identification of the Chido/Rodgers (Ch/Rg) blood groups. Researchers discovered that these blood groups were associated with different forms of the C4 protein. Further studies revealed that C4A and C4B are expressed on the membranes of erythrocytes and are linked to the HLA system. This discovery highlighted the genetic complexity of C4 and its role in the immune system .

Clinical Relevance

Measuring the levels of C4 in the blood can provide valuable information about an individual’s immune status. Low levels of C4 may indicate an underlying immune deficiency or autoimmune disorder. Therefore, C4 tests are often used in clinical settings to diagnose and monitor these conditions .

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