Furmaniak J, Kominami S, Asawa T, Wedlock N, Colls J, Smith B R
Department of Medicine, University of Wales College of Medicine, Cardiff, UK.
J Clin Endocrinol Metab. 1994 Nov;79(5):1517-21. doi: 10.1210/jcem.79.5.7962352.
Autoantibodies to steroid 21-hydroxylase (21-OH) are characteristic of adult onset Addison's disease and we have investigated the effects of these autoantibodies on recombinant human 21-OH enzyme activity. Antibody preparations from 11/11 Addison sera inhibited the ability of 21-OH to convert progesterone to deoxycorticosterone with 8 IgGs showing almost complete inhibition, 2 partial inhibition and 1 weak inhibition. Control IgGs from patients with autoimmune thyroid disease and normal blood donors had little or no effect on 21-OH activity. Our results suggest that 21-OH autoantibodies have the potential to contribute to adrenal failure in Addison's disease by inhibiting the 21-OH enzyme.
针对类固醇21-羟化酶（21-OH）的自身抗体是成人发病型艾迪生病的特征，我们研究了这些自身抗体对重组人21-OH酶活性的影响。11份艾迪生病血清的抗体制剂抑制了21-OH将孕酮转化为脱氧皮质酮的能力，其中8份免疫球蛋白显示几乎完全抑制，2份部分抑制，1份弱抑制。来自自身免疫性甲状腺疾病患者和正常献血者的对照免疫球蛋白对21-OH活性几乎没有影响。我们的结果表明，21-OH自身抗体有可能通过抑制21-OH酶导致艾迪生病中的肾上腺功能衰竭。
