The pulmonary alveolar proteinosis in granulocyte macrophage colony-stimulating factor/interleukins 3/5 beta c receptor-deficient mice is reversed by bone marrow transplantation
The pulmonary alveolar proteinosis in granulocyte macrophage colony-stimulating factor/interleukins 3/5 beta c receptor-deficient mice is reversed by bone marrow transplantation
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DOI：
10.1084/jem.183.6.2657
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发表时间：
1996-06-01
影响因子：
15.3
通讯作者：
Murray, R
中科院分区：
文献类型：
--
作者：
Nishinakamura, R;Wiler, R;Murray, R
粒细胞巨噬细胞集落刺激因子（GM-CSF）或GM-CSF共同受体组分（β - c）、白细胞介素(IL)-3和IL-5突变的小鼠表现出一种肺部疾病，类似于人类肺泡蛋白沉积症，这是一种先天性、婴儿和成人形式的罕见疾病。骨髓移植和野生型骨髓的β c突变小鼠的造血重建逆转了肺部既定的疾病状态，将这种疾病定义为造血疾病。该疾病可能与肺泡巨噬细胞有关，因为将供体骨髓细胞植入突变受体小鼠的肺部与疾病的恢复以及在受影响动物的肺部观察到的异常巨噬细胞形态相关。重组激活基因2突变供体骨髓缺乏发展淋巴细胞的潜力，在与整个骨髓相同的程度上逆转了肺部的病理。这些数据表明，某些肺疾病，如果是细胞自主造血起源，可以通过骨髓移植来控制。
Mice mutant for granulocyte macrophage colony-stimulating factor (GM-CSF) or the common receptor component (beta c) for GM-CSF, interleukin (IL)-3, and IL-5 exhibit a lung disorder Similar to human pulmonary alveolar proteinosis, a rare disease with congenital, infantile, and adult forms. Bone marrow transplantation and hematopoietic reconstitution of beta c mutant mice with wild-type bone marrow reversed the established disease state in the lungs, defining this disease as hematopoietic in nature. It is likely that the disease involves alveolar macrophages, as donor myeloid cell engraftment into the lungs of mutant recipient mice correlated with reverting both the disease and an abnormal macrophage morphology seen in the lungs of affected animals. Recombination Activating Gene-2 mutant donor bone marrow, which lacks the potential to develop lymphocytes, reversed the pathology in the lungs to the same extent as whole bone marrow. These data establish that certain lung disorders, if of cell-autonomous hematopoietic origin, can be manipulated by bone marrow transplantation.