Clemente M G, Meloni A, Obermayer-Straub P, Frau F, Manns M P, De Virgiliis S
Istituto di Clinica e Biologia dell'età evolutiva, Università degli Studi di Cagliari, Italy.
Gastroenterology. 1998 Feb;114(2):324-8. doi: 10.1016/s0016-5085(98)70484-6.
BACKGROUND & AIMS: Liver disease has been described in 10%-15% of patients with autoimmune polyglandular syndrome type 1 (APS-1). After the discovery of cytochrome P450 1A2 (CYP1A2) as a hepatocellular autoantigen in liver-kidney microsomal autoantibody (LKM)-positive patients with APS-1, the investigation of antiliver antibodies was extended to 11 Sardinian patients with APS-1.
Indirect immunofluorescence and Western blotting analysis were performed to study the antiliver antibodies.
Immunofluorescence revealed LKM antibodies in 3 patients with APS-1, 1 of whom died of fulminant hepatitis. Western blotting showed a liver microsomal protein band of approximately 51 kilodaltons in the LKM-positive sera of these 3 patients. Western blotting performed with recombinant cytochrome P450 enzymes allowed the identification of CYP2A6 as a specific target antigen.
LKM antibodies in APS-1 sera are specifically directed against CYP1A2 or CYP2A6, but their diagnostic and prognostic significance for liver disease remain to be determined.
10%-15%的1型自身免疫性多腺体综合征（APS-1）患者存在肝脏疾病。在发现细胞色素P450 1A2（CYP1A2）是APS-1相关的肝肾微粒体自身抗体（LKM）阳性患者的肝细胞自身抗原后，对11名撒丁岛APS-1患者的抗肝脏抗体研究得以扩展。
采用间接免疫荧光和蛋白质印迹分析来研究抗肝脏抗体。
免疫荧光显示3例APS-1患者存在LKM抗体，其中1例死于暴发性肝炎。蛋白质印迹显示这3例LKM阳性血清中存在一条约51千道尔顿的肝脏微粒体蛋白条带。用重组细胞色素P450酶进行蛋白质印迹可鉴定出CYP2A6为特异性靶抗原。
APS-1血清中的LKM抗体特异性针对CYP1A2或CYP2A6，但其对肝脏疾病的诊断和预后意义仍有待确定。
