Nicoloff G, Angelova M, Nikolov A
Division of Biology, Department of Biology and Pathological Physiology, University School of Medicine, 1, St. Kliment Ohridski Street, 5800 Pleven, Bulgaria.
Vascul Pharmacol. 2005 Sep;43(3):171-5. doi: 10.1016/j.vph.2005.06.005.
The fibrillins are large glycoproteins components of 10-nm microfibrils found in the extracellular matrix of most tissues. Microfibrils play a role in elastic fiber assembly and serve to link cells to elastic fibers in the extracellular matrix. Fibrillin-1 (FBN-1) and -2 (FBN-2) are large, secreted glycoproteins known to be components of extracellular matrix microfibrils located in the vasculature, basement membrane, and various connective tissues and are often associated with a superstructure known as the elastic fiber. Anti-fibrillin antibodies found in some autoimmune diseases could form circulating immune complexes (CIC) with corresponding antigens. Type 1 (insulin-dependent) diabetes mellitus is an autoimmune disease leading to formation of different types of autoantibodies. To determine the possible presence of FBN-anti-FBN CIC (IgG and IgM) were studied by modified version of ELISA 35 children with Type 1 diabetes mellitus (mean age--12.37+/-3.77 years, diabetes duration 4+/-3.5 years). Eight of the diabetics had vascular complications. Twenty healthy children (mean age--11.58+/-2.89 years) were used as controls. Diabetics showed statistically significant higher levels of FBN-anti-FBN-2 CIC - IgG (0.303+/-0.076 vs. 0.252+/-0.029; p=0.029) and IgM (0.415+/-0.085 vs. 0.348+/-0.069; p=0.018) compared to the control group. FBN-anti-FBN-1 CIC IgM correlate with diabetes duration (r=0.52; p=0.0015) and BMI (r=0.33, p=0.053) while FBN-anti-FBN-1 CIC IgG correlate with serum Zinc (r=0.49, p=0.006). FBN-anti-FBN-2 CIC IgG correlate with microalbuminuria (r=0.65, p=0.0046) and retinopathy (r=0.61, p=0.0001). This study suggests that there may be a relationship of levels of FBN-anti-FBN-2 CIC IgG with the development of diabetic microangiopathy. Of course the number of the tested patients is limited for definitive conclusions. Although the meaning of these results is still being determined, the measurement of FBN-anti-FBN CIC may represent immunologic markers of FBN metabolism.
原纤蛋白是大多数组织细胞外基质中10纳米微原纤维的大型糖蛋白成分。微原纤维在弹性纤维组装中发挥作用，并将细胞与细胞外基质中的弹性纤维相连。原纤蛋白-1（FBN-1）和-2（FBN-2）是大型分泌性糖蛋白，已知是位于脉管系统、基底膜和各种结缔组织中的细胞外基质微原纤维的成分，常与一种称为弹性纤维的超结构相关。在一些自身免疫性疾病中发现的抗原纤蛋白抗体可与相应抗原形成循环免疫复合物（CIC）。1型（胰岛素依赖型）糖尿病是一种导致形成不同类型自身抗体的自身免疫性疾病。为确定FBN-抗FBN CIC（IgG和IgM）的可能存在情况，采用改良版ELISA对35例1型糖尿病患儿（平均年龄12.37±3.77岁，糖尿病病程4±3.5年）进行了研究。其中8例糖尿病患儿有血管并发症。选取20名健康儿童（平均年龄11.58±2.89岁）作为对照。与对照组相比，糖尿病患儿的FBN-抗FBN-2 CIC - IgG（0.303±0.076对0.252±0.029；p=0.029）和IgM（0.415±0.085对0.348±0.069；p=0.018）水平在统计学上显著更高。FBN-抗FBN-1 CIC IgM与糖尿病病程（r=0.52；p=0.0015）和BMI（r= .33，p=0.053）相关，而FBN-抗FBN-1 CIC IgG与血清锌（r=0.49，p=0.006）相关。FBN-抗FBN-2 CIC IgG与微量白蛋白尿（r=0.65，p=0.0046）和视网膜病变（r=0.61，p=0.0001）相关。本研究表明，FBN-抗FBN-2 CIC IgG水平可能与糖尿病微血管病变的发生有关。当然，由于受试患者数量有限，无法得出确定性结论。尽管这些结果的意义仍在确定中，但FBN-抗FBN CIC的检测可能代表原纤蛋白代谢的免疫标志物。
