Lavoie Tegan N, Lee Byung Ha, Nguyen Cuong Q
Department of Oral Biology, College of Dentistry, University of Florida, P.O. Box 100424, 1600 SW Archer Road, Gainesville, FL 32610, USA.
J Biomed Biotechnol. 2011;2011:549107. doi: 10.1155/2011/549107. Epub 2010 Dec 30.
Sjögren's syndrome (SjS) is a complex chronic autoimmune disease of unknown etiology which primarily targets the exocrine glands, resulting in eventual loss of secretory function. The disease can present as either primary SjS or secondary SjS, the latter of which occurs concomitantly with another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or primary biliary cirrhosis. Current advancements in therapeutic prevention and treatment for SjS are impeded by lack of understanding in the pathophysiological and clinical progression of the disease. Development of appropriate mouse models for both primary and secondary SjS is needed in order to advance knowledge of this disease. This paper details important features, advantages, and pitfalls of current animal models of SjS, including spontaneous, transgenic, knockout, immunization, and transplantation chimera mouse models, and emphasizes the need for a better model in representing the human SjS phenotype.
干燥综合征(SjS)是一种病因不明的复杂慢性自身免疫性疾病,主要累及外分泌腺,最终导致分泌功能丧失。该疾病可表现为原发性干燥综合征或继发性干燥综合征,后者与另一种自身免疫性疾病如类风湿关节炎、系统性红斑狼疮、硬皮病或原发性胆汁性肝硬化同时发生。由于对该疾病的病理生理和临床进展缺乏了解,目前干燥综合征治疗预防方面的进展受到阻碍。为了增进对这种疾病的了解,需要开发适用于原发性和继发性干燥综合征的小鼠模型。本文详细介绍了当前干燥综合征动物模型的重要特征、优点和缺陷,包括自发、转基因、基因敲除、免疫和移植嵌合小鼠模型,并强调需要一个能更好地呈现人类干燥综合征表型的模型。