Human cysteine proteinase inhibitors. Isolation, physiological importance, inhibitory mechanism, gene structure and relation to hereditary cerebral hemorrhage.
Human cysteine proteinase inhibitors. Isolation, physiological importance, inhibitory mechanism, gene structure and relation to hereditary cerebral hemorrhage.
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人半胱氨酸蛋白酶抑制剂。
DOI：
10.1080/00365518809168291
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
Magnus Abrahamson
中科院分区：
文献类型：
--
作者：
Magnus Abrahamson
本文报道了六种人半胱氨酸蛋白酶抑制剂的分离和鉴定。并从生理功能方面对它们在人体体液中的分布进行了描述和讨论。对激肽原和半胱氨酸蛋白酶C的结构-功能关系进行了研究，并根据半胱氨酸蛋白酶抑制剂C的研究结果，提出了半胱氨酸蛋白酶抑制机制的一般模型。利用该模型构建了对木瓜酶和链球菌半胱氨酸蛋白酶有良好抑制作用的合成抑制剂。介绍了正常人胱抑素C基因的结构，以及遗传性胱抑素C淀粉样血管病患者的胱抑素C基因的研究。结果，发现了一个显示与该病完全共分离的RFLP。结论：本病是由半胱氨酸氨基转移酶C结构基因点突变引起的，RFLP是诊断HCCAA最有用的工具。还报道了在大肠杆菌中生产重组半胱氨酸氨基转移酶C的方法，并讨论了其用于治疗HCCAA的可能性。
The isolation and characterization of six human cysteine proteinase inhibitors is reported. Their distribution in human biological fluids is also described and discussed with respect to physiological function. Studies on kininogen and cystatin C with respect to structure-function relationships and, as a result of the cystatin C studies, a general model for the mechanism of cysteine proteinase inhibition by cystatins are presented. The model was used for the construction of synthetic inhibitors which showed good inhibitory properties against papain and the streptococcal cysteine proteinase. Structures of cDNA and gene for normal human cystatin C are accounted for, as well as studies on the cystatin C gene in patients suffering from hereditary cystatin C amyloid angiopathy (HCCAA). As a result of this an RFLP that showed total co-segregation with the disease was found. It was concluded that the disease is caused by a point mutation in the cystatin C structural gene and that the RFLP will be a most useful tool for diagnosis of HCCAA. The production of recombinant cystatin C in E. coli is also reported and its possible use for treatment of HCCAA is discussed.
α2-硫醇蛋白酶抑制剂的人 cDNA 的分离及其与低分子量激肽原的同一性。
影响因子：
2.9
作者：
Ohkubo,I;Kurachi,K;Takasawa,T;Shiokawa,H;Sasaki,M
通讯作者：
Sasaki,M