• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利：CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法，用中文像聊天一样搜索，搜遍4000万医学文献。AI智能推荐，让科研检索更轻松。

立即免费搜索

文件翻译

保留排版，准确专业，支持PDF/Word/PPT等文件格式，支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述，25分钟生成高质量综述，智能提取关键信息，辅助科研写作。

立即免费体验

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.

DOI:10.1084/jem.147.4.1285
PMID:650154
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2184240/
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。

相似文献

1
Specific protease deficiency in polymorphonuclear leukocytes of Chédiak-Higashi syndrome and beige mice.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。
J Exp Med. 1978 Apr 1;147(4):1285-90. doi: 10.1084/jem.147.4.1285.
2
Lysosomal elastase and cathepsin G in beige mice. Neutrophils of beige (Chediak-Higashi) mice selectively lack lysosomal elastase and cathepsin G.米色小鼠中的溶酶体弹性蛋白酶和组织蛋白酶G。米色（切迪阿克-东综合征）小鼠的中性粒细胞选择性缺乏溶酶体弹性蛋白酶和组织蛋白酶G。
J Exp Med. 1986 Mar 1;163(3):665-77. doi: 10.1084/jem.163.3.665.
3
Elastase and cathepsin G activities are present in immature bone marrow neutrophils and absent in late marrow and circulating neutrophils of beige (Chediak-Higashi) mice.弹性蛋白酶和组织蛋白酶G的活性存在于未成熟的骨髓中性粒细胞中，而在米色（切迪阿克-希加什）小鼠的晚期骨髓和循环中性粒细胞中则不存在。
J Exp Med. 1987 Nov 1;166(5):1362-76. doi: 10.1084/jem.166.5.1362.
4
Inhibitors of elastase and cathepsin G in Chédiak-Higashi (beige) neutrophils.嗜酸性粒细胞增多症（米色）中性粒细胞中弹性蛋白酶和组织蛋白酶G的抑制剂
J Biol Chem. 1989 May 5;264(13):7431-6.
5
Enhanced diacylglycerol production by phospholipase D activation is responsible for abnormal increase in concanavalin A cap formation in polymorphonuclear leukocytes from Chediak-Higashi syndrome (beige) mice.磷脂酶D激活导致的二酰甘油生成增强，是切-东综合征（米色）小鼠多形核白细胞中伴刀豆球蛋白A帽形成异常增加的原因。
Int Immunopharmacol. 2014 Jul;21(1):193-9. doi: 10.1016/j.intimp.2014.05.003. Epub 2014 May 14.
6
A thiol proteinase inhibitor, E-64-d, corrects the abnormalities in concanavalin A cap formation and the lysosomal enzyme activity in leucocytes from patients with Chediak-Higashi syndrome by reversing the down-regulated protein kinase C activity.一种巯基蛋白酶抑制剂E-64-d，通过逆转下调的蛋白激酶C活性，纠正了切迪阿克-东综合征患者白细胞中伴刀豆球蛋白A帽形成和溶酶体酶活性的异常。
Clin Exp Immunol. 2001 Aug;125(2):283-90. doi: 10.1046/j.1365-2249.2001.01598.x.
7
Immunologic vasculitis in beige mice with deficiency of leukocytic neutral protease.白细胞中性蛋白酶缺乏的米色小鼠中的免疫性血管炎
J Immunol. 1979 May;122(5):1807-11.
8
Neutrophils in beige mice secrete normal amounts of cathepsin G and a 46 kDa latent form of elastase that can be activated extracellularly by proteolytic activity.米色小鼠中的中性粒细胞分泌正常量的组织蛋白酶G和一种46 kDa的潜伏形式的弹性蛋白酶，该弹性蛋白酶可通过蛋白水解活性在细胞外被激活。
Biol Chem. 1997 May;378(5):417-23. doi: 10.1515/bchm.1997.378.5.417.
9
Normal-sized primary lysosomes are present in Chediak-Higashi syndrome neutrophils.在切-东综合征中性粒细胞中存在正常大小的初级溶酶体。
Pediatr Res. 1987 Aug;22(2):208-15. doi: 10.1203/00006450-198708000-00022.
10
Giant granules of neutrophils in Chediak-Higashi syndrome are derived from azurophil granules but not from specific and gelatinase granules.切迪阿克-东综合征中嗜中性粒细胞的巨大颗粒源自嗜天青颗粒，而非特异性颗粒和明胶酶颗粒。
J Leukoc Biol. 1998 Jul;64(1):72-7. doi: 10.1002/jlb.64.1.72.

引用本文的文献

1
Elevated plasma interleukin-6 levels in trained male triathletes following an acute period of intense interval training.在经过一段急性期的高强度间歇训练后，训练有素的男性铁人三项运动员血浆白细胞介素-6水平升高。
Eur J Appl Physiol. 2007 Mar;99(4):353-60. doi: 10.1007/s00421-006-0354-y. Epub 2006 Dec 13.
2
A thiol proteinase inhibitor, E-64-d, corrects the abnormalities in concanavalin A cap formation and the lysosomal enzyme activity in leucocytes from patients with Chediak-Higashi syndrome by reversing the down-regulated protein kinase C activity.一种巯基蛋白酶抑制剂E-64-d，通过逆转下调的蛋白激酶C活性，纠正了切迪阿克-东综合征患者白细胞中伴刀豆球蛋白A帽形成和溶酶体酶活性的异常。
Clin Exp Immunol. 2001 Aug;125(2):283-90. doi: 10.1046/j.1365-2249.2001.01598.x.
3
Neutrophil lysosomal dysfunctions in mutant C57 Bl/6J mice: interstrain variations in content of lysosomal elastase, cathepsin G and their inhibitors.突变型C57 Bl/6J小鼠中性粒细胞溶酶体功能障碍：溶酶体弹性蛋白酶、组织蛋白酶G及其抑制剂含量的品系间差异
Biochem J. 1994 Apr 1;299 ( Pt 1)(Pt 1):237-45. doi: 10.1042/bj2990237.
4
Nonspecific protease and elastase activities in rat leukocytes.大鼠白细胞中的非特异性蛋白酶和弹性蛋白酶活性
Inflammation. 1982 Jun;6(2):177-87. doi: 10.1007/BF00916242.
5
Target-effector cell interaction in the natural killer cell system. V. Energy requirements, membrane integrity, and the possible involvement of lysosomal enzymes.自然杀伤细胞系统中的靶效应细胞相互作用。V. 能量需求、膜完整性以及溶酶体酶的可能参与。
Immunology. 1980 May;40(1):107-16.
6
Chédiak-Higashi gene in humans. II. The selectivity of the defect in natural-killer and antibody-dependent cell-mediated cytotoxicity function.人类的Chédiak-Higashi基因。II. 自然杀伤细胞和抗体依赖性细胞介导的细胞毒性功能缺陷的选择性
J Exp Med. 1980 May 1;151(5):1049-58. doi: 10.1084/jem.151.5.1049.
7
Tubulin tyrosinolation in human polymorphonuclear leukocytes: studies in normal subjects and in patients with the Chediak-Higashi syndrome.人类多形核白细胞中的微管蛋白酪氨酸化：对正常受试者和患有切-希二氏综合征患者的研究
J Cell Biol. 1982 Nov;95(2 Pt 1):519-26. doi: 10.1083/jcb.95.2.519.
8
Listeriosis in beige mice and their heterozygous littermates.米色小鼠及其杂合子同窝仔鼠中的李斯特菌病。
Immunology. 1984 Apr;51(4):711-7.
9
The effect of the beige mutation on infection with murine cytomegalovirus: histopathologic studies.米色突变对小鼠巨细胞病毒感染的影响：组织病理学研究。
Am J Pathol. 1982 Sep;108(3):299-309.
10
Pathogenesis of murine cytomegalovirus infection in natural killer cell-depleted mice.自然杀伤细胞缺失小鼠中鼠巨细胞病毒感染的发病机制
J Virol. 1984 Oct;52(1):119-28. doi: 10.1128/JVI.52.1.119-128.1984.

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.
10
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.