Formiminotransferase Cyclodeaminase (LC1)/亚胺甲基转移酶环脱氨酶

Formiminotransferase Cyclodeaminase (LC1)

Diseases:
Autoimmune Hepatitis Type II
 

Formiminotransferase cyclodeaminase, a bifunctional enzyme of tetrahydrofolate synthesis, is the target antigen of anti-LC1 (liver cytosol antigen type 1) autoantibodies. Presence of LC1 autoantibodies is a marker for type 2 autoimmune hepatitis (for which anti-LKM 1/cytochrome P450 2D6 autoantibodies are a further marker).

Serum LC1 autoantibody concentrations appear to fluctuate in parallel with aminotransferase levels, a particularly intriguing observation that suggests a possible role of LC1 autoreactivity in the pathogenic mechanism leading to hepatocyte injury.

Full-length formiminotransferase cyclodeaminase from DIARECT is produced in the baculovirus expression system.

相关疾病：自身免疫性肝炎II 型身免疫性肝炎（AIH）是一种病因不明并且对肝薄壁组织逐渐损害的紊乱性疾病。如果不进行治疗会有很高的死亡率。该疾病的特点之一是在大约90%以上患该病的患者血清中可检出自身抗体。从临床和血清学角度，把AIH病人分为两种不同的类型。Ⅰ型的特点是在AIH患者血清中可检测到抗平滑肌抗体和/或抗核酸抗体，Ⅱ型的病人可检测出抗肝肾微粒体1型抗体（LKM1）和抗肝细胞胞质1型抗体（LC1）。 LC1自身抗体在10%的AIH病人血清中可检测到，可作为一种血清学标记物。亚胺甲基转移酶是四氢叶酸酯合成的双功能酶，是抗LC1（肝细胞胞质抗原1型）自身抗体的抗原靶标。LC1自身抗体的存在是自身免疫肝炎2型的标志物（抗LKM1或细胞色素P450 2D6自身抗体是进一步的标记物）血清中LC1自身抗体的浓度与转氨酶水平平行波动，一项独特的有趣的观察发现，LC1的自身反应性在致病机制中可能的作用可能会导致肝损伤。

       DIARECT生产的全长亚胺甲基转移酶环化脱氨酶是在杆状病毒表达系统表达的。