Sköldberg Filip, Rorsman Fredrik, Perheentupa Jaakko, Landin-Olsson Mona, Husebye Eystein S, Gustafsson Jan, Kämpe Olle
Department of Medical Sciences, Uppsala University, University Hospital, 751 85 Uppsala, Sweden.
J Clin Endocrinol Metab. 2004 Apr;89(4):1636-40. doi: 10.1210/jc.2003-031161.
The structurally related group II pyridoxal phosphate (PLP)-dependent amino acid decarboxylases glutamic acid decarboxylase (GAD), aromatic L-amino acid decarboxylase (AADC), and histidine decarboxylase (HDC) are known autoantigens in endocrine disorders. We report, for the first time, the prevalence of serum autoantibody reactivity against cysteine sulfinic acid decarboxylase (CSAD), an enzyme that shares 50% amino acid identity with the 65- and 67-kDa isoforms of GAD (GAD-65 and GAD-67), in endocrine autoimmune disease. Three of 83 patients (3.6%) with autoimmune polyendocrine syndrome type 1 (APS1) were anti-CSAD positive in a radioimmunoprecipitation assay. Anti-CSAD antibodies cross-reacted with GAD-65, and the anti-CSAD-positive sera were also reactive with AADC and HDC. The low frequency of anti-CSAD reactivity is in striking contrast to the prevalence of antibodies against GAD-65, AADC, and HDC in APS1 patients, suggesting that different mechanisms control the immunological tolerance toward CSAD and the other group II decarboxylases. Moreover, CSAD may be a useful mold for the construction of recombinant chimerical antigens in attempts to map conformational epitopes on other group II PLP-dependent amino acid decarboxylases.
结构相关的II组磷酸吡哆醛(PLP)依赖性氨基酸脱羧酶,即谷氨酸脱羧酶(GAD)、芳香族L-氨基酸脱羧酶(AADC)和组氨酸脱羧酶(HDC),是内分泌疾病中已知的自身抗原。我们首次报告了在内分泌自身免疫性疾病中,血清针对半胱氨酸亚磺酸脱羧酶(CSAD)的自身抗体反应性的患病率,CSAD是一种与GAD的65 kDa和67 kDa亚型(GAD-65和GAD-67)具有50%氨基酸同一性的酶。在放射免疫沉淀试验中,83例1型自身免疫性多内分泌综合征(APS1)患者中有3例(3.6%)抗CSAD呈阳性。抗CSAD抗体与GAD-65发生交叉反应,抗CSAD阳性血清也与AADC和HDC发生反应。抗CSAD反应性的低频率与APS1患者中抗GAD-65、AADC和HDC抗体的患病率形成鲜明对比,这表明不同的机制控制着对CSAD和其他II组脱羧酶的免疫耐受性。此外,CSAD可能是构建重组嵌合抗原的有用模板,以试图绘制其他II组PLP依赖性氨基酸脱羧酶上的构象表位。