Immunological characterization of (tight skin/NZB)F1 hybrid mice with connective tissue and autoimmune features resembling human systemic sclerosis.
Immunological characterization of (tight skin/NZB)F1 hybrid mice with connective tissue and autoimmune features resembling human systemic sclerosis.
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具有结缔组织和类似于人类系统性硬化症的自身免疫特征的（紧皮肤/NZB）F1 杂交小鼠的免疫学特征。
DOI：
10.1006/jaut.1993.1029
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发表时间：
1993
影响因子：
12.8
通讯作者：
Jimenez,SA
中科院分区：
文献类型：
--
作者：
Bocchieri,MH;Christner,PJ;Henriksen,PD;Jimenez,SA
通过将TSK+/+ pa紧皮小鼠（TSK）和自身免疫性疾病易感的NZB品系繁殖以产生F1杂交体，该杂交体显示TSK亲本的结缔组织异常和NZB亲本的自身免疫异常，已经开发了一种新的系统性硬化症（SSc）小鼠模型。（TSK/NZB）F1代的肩胛间皮肤厚度显著大于（+pa/NZB）F1代同窝仔。皮肤穿刺活检中的蛋白质生物合成在（TSK/NZB）F1小鼠中比对照组高3.5倍以上。羟脯氨酸分析证实，蛋白质合成的增加主要是在胶原蛋白。对这些（TSK/NZB）F1小鼠进行了大量细胞和体液自身免疫表现的检测。自身抗体，包括抗核抗体（ANA）和抗DNA，存在于他们的血清中，和自体淋巴细胞刺激（AMLR）的增殖反应降低，是常见的小鼠和人类自身免疫性疾病。这些结果表明，（TSK/NZB）F1小鼠显示结缔组织和免疫异常类似于那些存在于人类SSc，因此，这些小鼠可能是一个有价值的模型，用于疾病的研究。
A new murine model of Systemic Sclerosis (SSc) has been developed by breeding the Tsk+/+ pa tight skin mouse (TSK) and the autoimmune disease-prone NZB strain to produce an F1 hybrid displaying the connective tissue abnormalities of the TSK parent and the autoimmune abnormalities of the NZB parent. The interscapular skin thickness in the (TSK/NZB)F1 was significantly greater than in the (+pa/NZB)F1 litter mates. Protein biosynthesis in skin punch biopsies was over 3.5 times higher in the (TSK/NZB)F1 mice than in controls. Hydroxyproline analyses confirmed that the increase in protein synthesis was primarily in collagen. These (TSK/NZB)F1 mice were tested for a number of cellular and humoral autoimmune manifestations. Autoantibodies, including antinuclear antibodies (ANA) and anti-DNA, were present in their sera, and the proliferative response to autologous lymphocyte stimulation (AMLR) was decreased as is commonly observed in murine and human autoimmune disorders. These results indicate that the (TSK/NZB)F1 mice display connective tissue and immunologic abnormalities resembling those present in human SSc and, therefore, these mice may be a valuable model for the study of the disease.