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通过tsk骨髓和脾细胞将tsk皮肤纤维化过继转移至基因野生型受体。

Adoptive transfer of tsk skin fibrosis to +/+ recipients by tsk bone marrow and spleen cells.

作者信息

Walker M A, Harley R A, DeLustro F A, LeRoy E C

机构信息

Department of Medicine, Medical University of South Carolina, Charleston 29425.

出版信息

Proc Soc Exp Biol Med. 1989 Nov;192(2):196-200. doi: 10.3181/00379727-192-42979.

DOI:10.3181/00379727-192-42979
PMID:2813452
Abstract

The tight skin mouse (Tsk/+) is an autosomal dominant example of inherited fibrosis whose pathogenesis is unclear. Autoimmune phenomena have been described previously. This study demonstrates the adoptive transfer of skin fibrosis to lethally irradiated syngeneic +/+ recipients by the transplantation of both bone marrow and spleen cells. The recipient skin fibrosis was not associated with mast cell proliferation or degranulation as it is in the tsk/+ mouse, representing for the first time a dissociation between mast cells and fibrosis and introducing the potential for direct T cell-fibroblast interactions.

摘要

紧皮小鼠(Tsk/+)是遗传性纤维化的常染色体显性遗传实例,其发病机制尚不清楚。先前已有自身免疫现象的描述。本研究证明,通过移植骨髓和脾细胞,可将皮肤纤维化过继转移至接受致死性照射的同基因+/+受体小鼠。受体小鼠的皮肤纤维化与tsk/+小鼠不同,不伴有肥大细胞增殖或脱颗粒,这首次表明肥大细胞与纤维化之间存在分离,并提示了T细胞与成纤维细胞直接相互作用的可能性。

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Adoptive transfer of tsk skin fibrosis to +/+ recipients by tsk bone marrow and spleen cells.通过tsk骨髓和脾细胞将tsk皮肤纤维化过继转移至基因野生型受体。
Proc Soc Exp Biol Med. 1989 Nov;192(2):196-200. doi: 10.3181/00379727-192-42979.
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Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome.原纤维蛋白-1 突变导致先天性硬皮病:硬皮病综合征。
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B-lymphocyte depletion reduces skin fibrosis and autoimmunity in the tight-skin mouse model for systemic sclerosis.
在系统性硬化症的紧皮小鼠模型中,B淋巴细胞耗竭可减轻皮肤纤维化和自身免疫反应。
Am J Pathol. 2006 Sep;169(3):954-66. doi: 10.2353/ajpath.2006.060205.
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Bone marrow transplantation in the treatment of systemic sclerosis.骨髓移植治疗系统性硬化症。
Curr Rheumatol Rep. 2000 Dec;2(6):492-500. doi: 10.1007/s11926-000-0026-6.
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Curr Rheumatol Rep. 1999 Oct;1(1):34-7. doi: 10.1007/s11926-999-0022-4.