切迪阿克-东综合征和米色小鼠多形核白细胞中的特异性蛋白酶缺乏。
Specific protease deficiency in polymorphonuclear leukocytes of Chédiak-Higashi syndrome and beige mice.
作者信息
Vassalli J D, Granelli-Piperno A, Griscelli C, Reich E
出版信息
J Exp Med. 1978 Apr 1;147(4):1285-90. doi: 10.1084/jem.147.4.1285.
Abstract
Peripheral blood leukocytes of three patients with Chédiak-Higashi syndrome (CHS) contained very low or undetectable levels of elastase, the major neutral protease in these cells. Likewise, peritoneal exudate leukocytes of beige mice (the murine counterpart of CHS) contained correspondingly reduced levels of their major neutral protease, a serine enzyme of mol wt 27,000. The elastase deficiency in CHS polymorphonuclear leukocytes might account in part for the high incidence of infections in these patients.
摘要
三名患有蔡迪二氏综合征（CHS）的患者外周血白细胞中，弹性蛋白酶（这些细胞中的主要中性蛋白酶）水平极低或无法检测到。同样，米色小鼠（CHS的鼠类对应物）的腹腔渗出白细胞中，其主要中性蛋白酶（一种分子量为27,000的丝氨酸酶）水平也相应降低。CHS多形核白细胞中的弹性蛋白酶缺乏可能部分解释了这些患者感染的高发生率。
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本文引用的文献
1
THE FAMILIAL OCCURRENCE OF THE CHEDIAK-HIGASHI SYNDROME IN MINK AND CATTLE.水貂和牛中切迪阿克-东综合征的家族性发生情况。
Genetics. 1964 Mar;49(3):505-12. doi: 10.1093/genetics/49.3.505.
2
In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood.人多形核白细胞的体外杀菌能力：儿童慢性肉芽肿病中活性降低。
J Clin Invest. 1967 Apr;46(4):668-79. doi: 10.1172/JCI105568.
3
Defective granulocyte regulation in the Chediak-Higashi syndrome.切-东综合征中粒细胞调节缺陷。
N Engl J Med. 1968 Nov 7;279(19):1009-15. doi: 10.1056/NEJM196811072791901.
4
The Chediak-Higashi syndrome: studies in four patients and a review of the literature.切-东综合征：4例患者的研究及文献综述
Medicine (Baltimore). 1972 Jul;51(4):247-80.
5
Phagocytosis in chronic granulomatous disease and the Chediak-Higashi syndrome.慢性肉芽肿病和切-东综合征中的吞噬作用。
N Engl J Med. 1972 Jan 20;286(3):120-3. doi: 10.1056/NEJM197201202860302.
6
Defective granulocyte chemotaxis in the Chediak-Higashi syndrome.切迪阿克-希加希综合征中粒细胞趋化性缺陷。
J Clin Invest. 1971 Dec;50(12):2645-52. doi: 10.1172/JCI106765.
7
Granulocyte function in the Chediak-Higashi syndrome of mice.小鼠柴-希二氏综合征中的粒细胞功能。
Blood. 1974 Feb;43(2):201-6.
8
Abnormal bactericidal, metabolic, and lysosomal functions of Chediak-Higashi Syndrome leukocytes.切-东综合征白细胞的杀菌、代谢及溶酶体功能异常。
J Clin Invest. 1972 Mar;51(3):649-65. doi: 10.1172/JCI106854.
9
The neutral proteases of human granulocytes. Isolation and partial characterization of two granulocyte collagenases.人粒细胞的中性蛋白酶。两种粒细胞胶原酶的分离及部分特性鉴定
Eur J Biochem. 1973 Jul 16;36(2):473-81. doi: 10.1111/j.1432-1033.1973.tb02932.x.
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The neutral proteases of human granulocytes. Isolation and partial characterization of granulocyte elastases.人粒细胞的中性蛋白酶。粒细胞弹性蛋白酶的分离及部分特性鉴定。
Eur J Biochem. 1974 Mar 1;42(2):519-27. doi: 10.1111/j.1432-1033.1974.tb03367.x.
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