A Lysosomal Storage Disorder in the BALB/c Mouse: Bone Marrow Transplantation
A Lysosomal Storage Disorder in the BALB/c Mouse: Bone Marrow Transplantation
复制标题
BALB/c 小鼠的溶酶体储存障碍：骨髓移植
DOI：
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发表时间：
1984
期刊：
影响因子：
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通讯作者：
R. Brady
中科院分区：
文献类型：
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作者：
A. Boothe;H. Weintroub;P. Pentchev;J. Jones;J. Butler;J. E. Barry;B. Neumeyer;J. Stivers;R. Brady
研究了将溶酶体储存障碍（BALB/c LSD）供体BALB/c小鼠的骨髓移植到正常受体小鼠的形态学和生化后果。在小鼠接受850拉德照射4小时后，从正常BALB/c和BALB/c LSD小鼠中取出骨髓输注到正常BALB/c受体小鼠中。240天后对受体小鼠进行组织检查。本研究发现，BALB/c LSD病变的缺陷细胞可以通过BALB/c LSD病变纯合子骨髓移植到正常BALB/c小鼠体内；BALB/c LSD缺陷细胞在受体单核吞噬系统中增殖和播散；受体组织中胆固醇、鞘脂和胱氨酸升高，鞘磷脂酶和葡萄糖脑苷酶活性降低；受者的寿命明显长于BALB/c LSD纯合子，其寿命主要受到辐照的二次效应的影响。这些病变虽然不像纯合子BALB/c LSD那样广泛，但与BALB/c LSD中发生的病变相似。由于受体小鼠没有受到BALB/c LSD小鼠寿命短（70天）的损害，因此它们可以用于研究这些代谢损伤的长期慢性影响。
The morphological and biochemical consequences of transplanting affected bone marrow from donor BALB/c mice with a lysosomal storage disorder (BALB/c LSD) into normal recipient mice were studied. Bone marrow was removed from normal BALB/c and BALB/c LSD mice and transfused into normal BALB/c recipient mice four hours after the mice received 850 rads of irradiation. Tissues of the recipient mice were examined 240 days later. This study revealed that the defective cells that constituted the visceral lesions of BALB/c LSD could be transplanted to normal BALB/c mice by the use of bone marrow from affected BALB/c LSD homozygote; that the defective cells of BALB/c LSD proliferated and disseminated throughout the mononuclear phagocytic system of the recipient; that there were increases in cholesterol, sphingolipids, and cystine with decreases in sphingomyelinase and glucocerebrosidase activity in tissues of the recipients; and that the recipients survived substantially longer than BALB/c LSD homozygotes and their lifespan was compromised mainly by the secondary effects of irradiation. These lesions, although not as extensive as in homozygous BALB/c LSD, paralleled the lesions which develop in BALB/c LSD. Since the the recipient mice were not compromised by the short life span (70 days) of the BALB/c LSD mice, they may be used to study the long-term chronic effects of these metabolic lesions.