本邦におけるキニノゲン欠損症の分子生物学的研究 第1編 キニノゲンに対するモノクローナル抗体による各ドメインの検索

Analysis of kininogen molecule status in four Japanese families affected with total kininogen deficiency and one Japanese family with isolated high molecular weight kininogen deficiency was performed using an immuoblotting method with monoclonal antibodies to high molecular weight kininogen (HKG H 12, L 7, L 17). The purified high molecular weight kininogen showed two bands with apparent mot wts of 120, 000 and 105, 000, respectively, and the purified low molecular weight kininogen showed a single band with an apparent mot wt of 60, 000. In normal human plasma, these three bands were also detected. No variant molecule of the kininogens was present in either cases of total kininogen deficiency or isolated high molecular weight kininogen deficiency.