摘要
再生障碍性贫血(aplastic anemia,AA)是一类以造血组织功能衰竭为特征的疾病,主要表现为骨髓造血功能低下及外周血全血细胞减少,包括先天性AA和获得性AA。先天性AA主要由于FA基因突变导致,而获得性AA,目前认为造血干细胞减少或缺损、造血微环境损伤以及T淋巴细胞功能亢进为该病的主要发病机制。针对不同发病机制建立AA动物模型,对于深入研究AA发病机制和选择有效的治疗靶点具有重要的临床意义。本文就先天性AA动物模型,诸如Fanc A-/-小鼠、Fanc C-/-小鼠、Fanc G-/-小鼠、Fanc D1-/-/Fanc 2-/-小鼠、Fanc D2-/-小鼠等及其他基因缺陷小鼠的AA模型,以及因造血干细胞减少、造血微环烷损伤、免疫介导及造血干细胞减少联合免疫介导所致的获得性AA模型进行综述。
Aplastic anemia （AA） is a disease, including congenital AA and acquired AA, characterized by an extremely hypocellular marrow and peripheral blood pancytopenia due to bone marrow failure. Congenital AA is a autosomal recessive disease due to gene mutation. Persently, acquired AA is recognized as a disease caused by destruction of hematopoietic stem cells, defective marrow rnicroenvironment and aberrant T cellular-immunity. In order to further study its pathogenesis and to choose effective therapeutic target, it has important clinical significance to establish correspondent animal model based on different pathogenesis. This article summarizes the congenital AA amimal models including Fanc A-/- mouse, Fanc C-/- mouse, Fanc G-/- mouse, Fanc D1-/-/Fanc 2-/- mouse, Fanc D2-/- mouse and other gene deficiency mouse AA models, and the acguired AA models resulting from the hematopoietic stem cell decrease, hematopoietic hematopoietic stem cell dicrease with immune mediation. t injury, immune mediation and combination of
出处
《中国实验血液学杂志》
CAS
CSCD
北大核心
2015年第1期285-289,共5页
Journal of Experimental Hematology
基金
国家自然科学基金(81041043)
天津市自然科学基金(13JCYBJC23400)
天津市卫生局科技基金攻关项目(2013KG11)
天津市卫生局科技基金重点项目(2013KR07)
