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先天性骨硬化症中的免疫功能:小眼畸形小鼠的淋巴细胞功能缺陷

Immune function in congenital osteopetrosis: defective lymphocyte function in microphthalmic mice.

作者信息

Minkin C, Trump G, Stohlman S A

出版信息

Dev Comp Immunol. 1982 Winter;6(1):151-9. doi: 10.1016/0145-305x(82)90017-9.

DOI:10.1016/0145-305x(82)90017-9
PMID:6978266
Abstract

Osteopetrosis is a prominent feature of a congenital mutation described in microphthalmic mice and is thought to be due to defective osteoclast function which causes a generalized lack of bone resorption. Reversal of defective bone resorption in osteopetrotic mutants has been achieved by hematopoietic cell transplantations; and conversely, defective bone resorption has been transferred to normals by hematopoietic cells from osteopetrotic littermates. This suggested that osteopetrotic mutants might also demonstrate defective immune functions which could in turn be related to the lack of normal bone resorption. To that end, aspects of in vitro lymphocyte function in microphthalmic mice were compared to those of their phenotypically normal littermates. There was a significant diminution in the proliferative response of splenocytes to mitogens in microphthalmic mice. Microphthalmic splenocytes also were less responsive in an in vitro assay which measured the capacity to form antibody forming cells.

摘要

骨硬化症是小眼畸形小鼠中一种先天性突变的显著特征,被认为是由于破骨细胞功能缺陷导致普遍缺乏骨吸收所致。通过造血细胞移植已实现骨硬化症突变体中缺陷性骨吸收的逆转;相反,来自骨硬化症同窝仔鼠的造血细胞已将缺陷性骨吸收转移至正常小鼠。这表明骨硬化症突变体可能也表现出免疫功能缺陷,而这反过来可能与正常骨吸收的缺乏有关。为此,将小眼畸形小鼠的体外淋巴细胞功能方面与其表型正常的同窝仔鼠进行了比较。小眼畸形小鼠的脾细胞对有丝分裂原的增殖反应显著降低。在一项测量形成抗体形成细胞能力的体外试验中,小眼畸形脾细胞的反应性也较低。

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1
Immune function in congenital osteopetrosis: defective lymphocyte function in microphthalmic mice.先天性骨硬化症中的免疫功能:小眼畸形小鼠的淋巴细胞功能缺陷
Dev Comp Immunol. 1982 Winter;6(1):151-9. doi: 10.1016/0145-305x(82)90017-9.
2
Immunological competence in osteopetrotic (ia) rats.骨石化(ia)大鼠的免疫能力
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J Exp Med. 1975 Sep 1;142(3):651-63. doi: 10.1084/jem.142.3.651.
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Immunological defect and its correction in the osteopetrotic mutant rat.骨石化突变大鼠的免疫缺陷及其纠正
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