21-hydroxylase, a major autoantigen in idiopathic Addison's disease

@article{Winqvist199221hydroxylaseAM,
  title={21-hydroxylase, a major autoantigen in idiopathic Addison's disease},
  author={Ola Winqvist and F. Anders Karlsson and Olle K{\"a}mpe},
  journal={The Lancet},
  year={1992},
  volume={339},
  pages={1559-1562}
}
Sera from patients with idiopathic Addison's disease commonly react with the zona glomerulosa of adrenal cortex. We used high-resolution western blot analysis of an adrenal microsomal fraction to investigate the target of these antibodies. A protein with an apparent molecular weight of 54 kDa was recognised as the common and major component. Sera identifying this autoantigen (from 12 of 16 patients) also showed strong immunofluorescence staining of a steroid-producing human adrenal… Expand
Autoantibody epitope mapping of the 21-hydroxylase antigen in autoimmune Addison's disease.
TLDR
The availability of recombinant antigens has permitted structural analysis of the autoepitopes involved in the autoimmune response to 21-hydroxylase in Addison's disease and these findings should lead to the development of a simple and specific tool for immunodiagnosis of the disease. Expand
Two different cytochrome P450 enzymes are the adrenal antigens in autoimmune polyendocrine syndrome type I and Addison's disease.
TLDR
The results show that the autoimmune responses towards adrenal tissue in patients suffering from APS I and Addison's disease are remarkably selective and suggest that a determination of the antigen involved in a patient with autoimmune adrenal insufficiency will have diagnostic as well as prognostic implications. Expand
Autoantibodies against 21-hydroxylase and side-chain cleavage enzyme in autoimmune Addison's disease are mainly immunoglobulin G1.
TLDR
The finding of a predominating IgG1 response against 21OH and SCC may suggest that T helper (Th) cells of the Th1 subclass are involved in destruction of the adrenal cortex in patients with autoimmune Addison's disease. Expand
The purification and application of biologically active recombinant steroid cytochrome P450 21-hydroxylase: the major autoantigen in autoimmune Addison's disease.
TLDR
It is concluded that the recombinant preparation of 21OH presented here is of sufficient purity and quality to be used for studies of cellular and humoral immunity in autoimmune Addison's disease. Expand
A conformation-dependent epitope in Addison's disease and other endocrinological autoimmune diseases maps to a carboxyl-terminal functional domain of human steroid 21-hydroxylase.
TLDR
It is concluded that the region involving R483 plays a key role in the formation of a three-dimensional epitope in a functionally important C-terminal domain of the enzyme 21-hydroxylase. Expand
The substrate-binding domain of 21-hydroxylase, the main autoantigen in autoimmune Addison's disease, is an immunodominant T cell epitope.
TLDR
21OH (342-361) is an immunodominant determinant for T cells in SJL and probably BALB/c mice and may be involved in the pathogenesis of autoimmune adrenal failure in humans. Expand
Expression of the addisonian autoantigen in human adrenal tumors.
TLDR
The data demonstrate that the expression of the Addisonian autoantigen correlates with the functional activity of adrenocortical neoplasms and suggest, that immunohistochemical stainings for steroid-producing enzymes may be clinically useful in the characterization of adrenal lesions. Expand
Cytochrome P450 1A2 is a hepatic autoantigen in autoimmune polyglandular syndrome type 1.
Autoantibodies directed against proteins of the adrenal cortex and the liver were studied in 88 subjects of Sardinian descent, namely six patients with autoimmune polyendocrine syndrome type 1Expand
Immunoprecipitation assay for autoantibodies to steroid 21-hydroxylase in autoimmune adrenal diseases.
TLDR
The studies indicate that the IPA provides a specific, sensitive, and convenient system for measuring 21-OH Abs, which is directed towards steroid 21-hydroxylase (21-OH; EC 1.99). Expand
Adrenal-cortex autoantibodies and steroid-producing cells autoantibodies in patients with Addison's disease: comparison of immunofluorescence and immunoprecipitation assays.
TLDR
A high association between these two markers in patients with different forms of autoimmune Addison's disease and in those with short- or long-standing disease was found; the only case negative for all these three markers suffered from Turner's syndrome. Expand
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