• 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

牛II型全身性糖原贮积病中的骨骼肌α-葡萄糖苷酶

Skeletal-muscle alpha-glucosidases in bovine generalized glycogenosis type II.

作者信息

Dorling P R, Howell J M, Gawthorne J M

出版信息

Biochem J. 1981 Aug 15;198(2):409-12. doi: 10.1042/bj1980409.

DOI:10.1042/bj1980409
PMID:7034730
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1163264/
Abstract

The skeletal muscle of cattle suffering from generalized glycogenosis type II was shown to lack acid alpha-glucosidase (EC 3.2.1.3) activity. Furthermore, there was no evidence of enzymically inactive proteins that cross-reacted with antibodies raised against acid alpha-glucosidase from the muscle of normal animals.

摘要

患II型全身性糖原贮积症牛的骨骼肌显示缺乏酸性α-葡萄糖苷酶(EC 3.2.1.3)活性。此外,没有证据表明存在与针对正常动物肌肉中酸性α-葡萄糖苷酶产生的抗体发生交叉反应的无酶活性蛋白。

相似文献

1
Skeletal-muscle alpha-glucosidases in bovine generalized glycogenosis type II.牛II型全身性糖原贮积病中的骨骼肌α-葡萄糖苷酶
Biochem J. 1981 Aug 15;198(2):409-12. doi: 10.1042/bj1980409.
2
Bovine generalised glycogenosis type II. Uptake of lysosomal alpha-glucosidase by cultured skeletal muscle and reversal of glycogen accumulation.
FEBS Lett. 1985 Oct 14;190(2):301-4. doi: 10.1016/0014-5793(85)81305-3.
3
Generalized glycogenosis in beef shorthorn cattle--heterozygote detection.
Aust J Exp Biol Med Sci. 1977 Apr;55(2):14U-50.
4
Uptake and stability of human and bovine acid alpha-glucosidase in cultured fibroblasts and skeletal muscle cells from glycogenosis type II patients.糖原贮积症II型患者培养的成纤维细胞和骨骼肌细胞中人和牛酸性α-葡萄糖苷酶的摄取与稳定性
Exp Cell Res. 1984 Nov;155(1):178-89. doi: 10.1016/0014-4827(84)90779-1.
5
Immunochemical studies of human acid alpha-1,4-glucosidase in type II glycogenosis.II型糖原贮积病中人类酸性α-1,4-葡糖苷酶的免疫化学研究。
Enzyme. 1981;26(4):182-90. doi: 10.1159/000459173.
6
Inhibition of bovine alpha-glucosidase by Castanospermum australe and its effect on the biochemical identification of heterozygotes for generalised glycogenosis type II (Pompe's disease) in cattle.澳大利亚栗豆树对牛α-葡萄糖苷酶的抑制作用及其对牛II型糖原累积病(庞贝氏病)杂合子生化鉴定的影响。
Aust Vet J. 1987 Sep;64(9):274-6. doi: 10.1111/j.1751-0813.1987.tb15956.x.
7
[Immunochemical study of acid alpha-1,4-glucosidase in 7 patients with type II glycogenosis].
Pediatrie. 1979 Sep;34(6):659-76.
8
Infantile and late onset form of generalised glycogenosis type II in cattle.牛的婴儿型和晚发型全身性II型糖原贮积病
J Pathol. 1981 Aug;134(4):267-77. doi: 10.1002/path.1711340403.
9
Bovine glycogenosis type II: the molecular defect in Shorthorn cattle.牛II型糖原贮积病:短角牛的分子缺陷
Neuromuscul Disord. 1994 Jan;4(1):39-48. doi: 10.1016/0960-8966(94)90046-9.
10
[Biochemical diagnosis of glycogenosis type II (acid maltase deficiency) (author's transl)].糖原贮积症Ⅱ型(酸性麦芽糖酶缺乏症)的生化诊断(作者译)
J Clin Chem Clin Biochem. 1977 Dec;15(12):705-8.

引用本文的文献

1
Preclinical Research in Glycogen Storage Diseases: A Comprehensive Review of Current Animal Models.糖原贮积病的临床前研究:当前动物模型的综合综述。
Int J Mol Sci. 2020 Dec 17;21(24):9621. doi: 10.3390/ijms21249621.
2
Hereditary neurovisceral mannosidosis associated with alpha-mannosidase deficiency in a family of Persian cats.与α-甘露糖苷酶缺乏相关的遗传性神经内脏甘露糖苷贮积症在一个波斯猫家族中出现。
Acta Neuropathol. 1982;58(1):64-8. doi: 10.1007/BF00692699.
3
Glycogen storage diseases in animals and their potential value as models of human disease.动物中的糖原贮积病及其作为人类疾病模型的潜在价值。
J Inherit Metab Dis. 1983;6(1):3-16. doi: 10.1007/BF02391186.

本文引用的文献

1
INBORN LYSOSOMAL DISEASES.先天性溶酶体病
Gastroenterology. 1965 May;48:625-33.
2
AN ELECTRON MICROSCOPIC AND BIOCHEMICAL STUDY OF TYPE II GLYCOGENOSIS.II型糖原贮积病的电子显微镜及生化研究
Lab Invest. 1964 Sep;13:1139-52.
3
alpha-Glucosidase deficiency in generalized glycogenstorage disease (Pompe's disease).全身性糖原贮积病(庞贝氏病)中的α-葡萄糖苷酶缺乏症。
Biochem J. 1963 Jan;86(1):11-6. doi: 10.1042/bj0860011.
4
Biochemical studies on a case of feline mannosidosis.一例猫甘露糖苷贮积症的生化研究
Biochem J. 1980 Sep 1;189(3):467-73. doi: 10.1042/bj1890467.
5
Acid alpha-D-glucosidase glucohydrolase from cattle liver.来自牛肝脏的酸性α-D-葡萄糖苷酶葡萄糖水解酶。
J Biol Chem. 1969 Sep 10;244(17):4735-42.
6
Rodent and human acid -glucosidase. Purification, properties and inhibition by antibodies. Investigation in type II glycogenosis.啮齿动物和人类的酸性葡萄糖苷酶。纯化、特性及抗体抑制作用。II型糖原贮积病的研究。
Eur J Biochem. 1972 Nov 21;31(1):156-65. doi: 10.1111/j.1432-1033.1972.tb02514.x.
7
Molecular studies on glycogen storage diseases.糖原贮积病的分子研究
Enzyme. 1974;18(1):60-72. doi: 10.1159/000459414.
8
Characterization of human liver alpha-D-mannosidase purified by affinity chromatography.通过亲和层析纯化的人肝脏α-D-甘露糖苷酶的特性分析。
Biochem J. 1976 Mar 1;153(3):579-87. doi: 10.1042/bj1530579.
9
Toward enzyme therapy for lysosomal storage diseases.迈向溶酶体贮积症的酶疗法。
Physiol Rev. 1976 Jan;56(1):57-99. doi: 10.1152/physrev.1976.56.1.57.
10
Characterization of the mutant alpha-mannosidase in bovine mannosidosis.牛甘露糖苷贮积症中突变α-甘露糖苷酶的特征分析
Biochem J. 1978 Dec 1;175(3):1013-22. doi: 10.1042/bj1751013.