The Journal of Clinical Endocrinology & Metabolism · 1994 · 51 citations · 6 references
Life SciencesBiochemistryEndocrinologyAutoimmunityHormone BiochemistrySteroid BiochemistryAdrenal EndocrinologyAdrenal HormonesAdrenal Steroid HormonesAdrenal SteroidogenesisAutoantibodiesAutoimmune Diseases21-Hydroxylase AntibodiesAdrenal Insufficiency SyndromesAdrenal PathophysiologyAdrenal Steroidogenesis DefectsAutoimmune Addison DiseaseAutoimmune Adrenal InsufficiencyDisorders Of Adrenal SteroidogenesisSteroidogenic Enzyme Defects
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.
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Takayuki Asawa, A Baumann-Antczak, B R Smith et al. · FEBS Letters · 1993 · 46 citations · Full text