THE LEUKOCYTE INTEGRINS
THE LEUKOCYTE INTEGRINS
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DOI：
10.1016/0167-5699(89)90238-7
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发表时间：
1989-04-01
期刊：
影响因子：
--
通讯作者：
HOGG, N
中科院分区：
文献类型：
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作者：
HOGG, N
其中B链的合成异常被公认！2..结果是，尽管在细胞的细胞质中可以检测到α L链（并且有时13条链），但没有白细胞整联蛋白α在膜上表达。目前已鉴定出约60例LAD患者，这些患者在I链基因中表现出5类突变，导致严重至中度的LAD疾病症状（D.安德森，休斯顿;。T. Kishimoto，斯坦福大学）。在已经详细分析的一种情况下，单个剪接位点突变导致90个碱基对外显子的缺失，该外显子定位于13链的胞外结构域（T. Kishimoto，斯坦福大学）。由于该13链在其他方面是正常的，因此该区域可能参与链连接。到目前为止，这种疾病的主要特点是在人，虽然一个左前降支狗有它的医疗费用照顾的感激研究工作者。人们希望，单个o~链的克隆和测序将提供一些理解的'混杂'的性质，
recognized in which synthesis of the B chain is abnorma! 2.. The result is that none of the leukocyte integrin~ are expressed on the membrane although oL chains (and sometimes 13 chains) can be detected in the cytoplasm of cells. About sixty LAD patients have now been identified showing five classes of mutation in the I~ chain gene that result in severe to moderate LAD disease symptoms (D. Anderson, Houston;. T. Kishimoto, Stanford). In one situation that has been analysed in detail, a single splice site mutation results in the deletion of a 90 base pair exon which maps to the extracellular domain of the 13 chain (T. Kishimoto, Stanford). Since this 13 chain is otherwise normal, it is possible that this region may be involved in chain linkage. To date, this disorder has been chiefly characterized in man although one LAD dog has had its medical expenses taken care of by grateful research workers. It was hoped that the cloning and sequencing of the individual o~ chains would provide some understanding of the'promiscuous' nature of