Absence of autoantibodies connected to autoimmune polyendocrine syndrome type I and II and Addison's disease in girls and women with Turner syndrome

Table 1

Disease	Antigen	Enzyme action
Addison's disease	21-Hydroxylase (21-OH)	Steroid hormone synthesis
Addison's disease with POF	21-Hydroxylase (21-OH)	Steroid hormone synthesis
	Side-chain cleavage enzyme (SCC)	Steroid hormone synthesis
APS I	17α-hydroxylase (17α-OH)	Steroid hormone synthesis
	Aromatic L-amino acid decarboxylase (AADC)	Monoaminergic and serotonergic biosynthetic pathways
	Tyrosine hydroxylase (TH)	Rate-limiting enzyme in catecholamine biosynthesis
	Tryptophan hydroxylase (TPH)	Rate limiting enzyme in the synthesis of serotonin
APS II	21-Hydroxylase (21-OH)	Steroid hormone synthesis

Description of important autoantigens in Addison's disease, Addison's disease with premature ovarian failure (POF), APS I and APS II. Patients with Addison's disease generally display autoantibodies to the enzyme 21-OH restricted to the adrenal cortex, while most of the patients with APS I show autoantibodies to SCC, located both in the adrenal cortex and steroid producing cells in the gonads, reflecting the risk of developing ovarian failure. In addition patients with Addison's disease and POF, also show antibodies directed against SCC (13). TPH is found as an intestinal autoantigen in APS I patients with intestinal dysfunction (14) and TH autoantibodies are correlated to alopecia areata in these patients (15).

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