Complement C4 deficient serum from guinea pig, for complement assays

Not available outside of the UK & Ireland.

Analysis Note

C4 is deficient as judged by a highly sensitive hemolytic assay and Ouchterlony immunodiffusion method.

Application

Complement C4 deficiency has long been associated with the disease systemic lupus erythematosus (SLE) and severe kidney disorders. Screening of the mutations which lead to C4 deficiency may assist in studies of these diseases and disorders. It has been demonstrated that 40-60% of patients with SLE contained either heterozygous or homozygous deficiencies in C4 compliment components via C4A and C4B genes. In particular, research has shown that complete C4A and C4B deficiencies occur through deleterious mutations at exon 13 and within a 2.6-kb genomic region spanning exons 20-29.