Dysfunctional C8 beta chain in patients with C8 deficiency.

摘要: Two sera from unrelated individuals, each lacking C8 activity, were examined by Western blot analysis. Using antisera raised against whole C8, the two are shown to lack beta chain, indicating a deficiency, which is frequently observed in cases of dysfunctional C8. In contrast, means specific anti-C8-beta antiserum, beta-like polypeptide chain apparently identical molecular weight compared normal was detected. Digestion and with Staphylococcus aureus V8 protease revealed distinct differences enzymatic digestion pattern. We conclude that dysfunction protein these patients resides this form deficiency deficiencies previously reported, one or both subunits lacking.