Kalliokoski Suvi, Piqueras Victoria Ortín, Frías Rafael, Sulic Ana-Marija, Määttä Juha A E, Kähkönen Niklas, Viiri Keijo, Huhtala Heini, Pasternack Arja, Laurila Kaija, Sblattero Daniele, Korponay-Szabó Ilma R, Mäki Markku, Caja Sergio, Kaukinen Katri, Lindfors Katri
Tampere Center for Child Health Research, School of Medicine, University of Tampere and Tampere University Hospital, PL 100, 33014, Tampere, Finland.
Department of Equine and Small Animal Medicine, Faculty of Veterinary Medicine, University of Helsinki, 00014, Helsinki, Finland.
Amino Acids. 2017 Mar;49(3):529-540. doi: 10.1007/s00726-016-2306-0. Epub 2016 Aug 9.
Coeliac disease is hallmarked by an abnormal immune reaction against ingested wheat-, rye- and barley-derived gluten and the presence of transglutaminase 2 (TG2)-targeted autoantibodies. The small-bowel mucosal damage characteristic of the disorder develops gradually from normal villus morphology to inflammation and finally to villus atrophy with crypt hyperplasia. Patients with early-stage coeliac disease have TG2-autoantibodies present in serum and small-intestinal mucosa and they may already suffer from abdominal symptoms before the development of villus atrophy. Previously, we have shown that intraperitoneal injections of coeliac patient-derived sera or purified immunoglobulin fraction into mice induce a condition mimicking early-stage coeliac disease. In the current study, we sought to establish whether recombinantly produced patient-derived TG2-targeted autoantibodies are by themselves sufficient for the development of such an experimentally induced condition in immune-compromised mice. Interestingly, mice injected with coeliac patient TG2-antibodies had altered small-intestinal mucosal morphology, increased lamina propria cellular infiltration and disease-specific autoantibodies deposited in the small bowel, but did not evince clinical features of the disease. Thus, coeliac patient-derived TG2-specific autoantibodies seem to be sufficient for the induction of subtle small-bowel mucosal alterations in mice, but the development of clinical features probably requires additional factors such as other antibody populations relevant in coeliac disease.
乳糜泻的特征是针对摄入的小麦、黑麦和大麦来源的麸质产生异常免疫反应,以及存在转谷氨酰胺酶2(TG2)靶向自身抗体。该疾病特征性的小肠黏膜损伤从正常绒毛形态逐渐发展为炎症,最终发展为伴有隐窝增生的绒毛萎缩。早期乳糜泻患者血清和小肠黏膜中存在TG2自身抗体,在绒毛萎缩出现之前他们可能就已经出现腹部症状。此前,我们已经表明,向小鼠腹腔注射乳糜泻患者来源的血清或纯化的免疫球蛋白组分可诱发一种类似于早期乳糜泻的病症。在本研究中,我们试图确定重组产生的患者来源的TG2靶向自身抗体本身是否足以在免疫受损小鼠中诱发这种实验性诱导的病症。有趣的是,注射了乳糜泻患者TG2抗体的小鼠小肠黏膜形态发生改变,固有层细胞浸润增加,并且在小肠中沉积了疾病特异性自身抗体,但未表现出该疾病的临床特征。因此,乳糜泻患者来源的TG2特异性自身抗体似乎足以在小鼠中诱导轻微的小肠黏膜改变,但临床特征的出现可能需要其他因素,如乳糜泻中相关的其他抗体群体。