Autoimmune Addison's disease--evidence for a role of steroid 21-hydroxylase autoantibodies in adrenal insufficiency.
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.
Furmaniak
J
J
Department of Medicine, University of Wales College of Medicine, Cardiff, UK.
Kominami
S
S
Asawa
T
T
Wedlock
N
N
Colls
J
J
Smith
B R
BR
D000224:Addison Disease / Q000209:etiology* / Q000276:immunology; D000309:Adrenal Insufficiency / Q000276:immunology*; D000328:Adult; D001323:Autoantibodies / Q000032:analysis / Q000276:immunology / Q000502:physiology*; D001327:Autoimmune Diseases / Q000209:etiology* / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D013255:Steroid 21-Hydroxylase / Q000276:immunology* / Q000502:physiology*
D001323:Autoantibodies; D007074:Immunoglobulin G; D013255:Steroid 21-Hydroxylase