正常人和葡糖神经酰胺贮积症患者皮肤成纤维细胞中膜结合葡糖神经酰胺：β-葡萄糖苷酶的激活 - Suppr

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Activation of membrane-bound glucosylceramide: beta-glucosidase in fibroblasts cultured from normal and glucosylceramidotic human skin.

作者信息

Mueller O T, Rosenberg A

出版信息

J Biol Chem. 1979 May 10;254(9):3521-5.

PMID:429368

Abstract

摘要

相似文献

Activation of membrane-bound glucosylceramide: beta-glucosidase in fibroblasts cultured from normal and glucosylceramidotic human skin.正常人和葡糖神经酰胺贮积症患者皮肤成纤维细胞中膜结合葡糖神经酰胺：β-葡萄糖苷酶的激活

J Biol Chem. 1979 May 10;254(9):3521-5.

The fate of glucosylceramide (glucocerebroside) in genetically impaired (lysosomal beta-glucosidase deficient) Gaucher disease diploid human fibroblasts.在基因受损（溶酶体β-葡萄糖苷酶缺乏）的戈谢病二倍体人成纤维细胞中葡萄糖神经酰胺（葡糖脑苷脂）的命运

J Biol Chem. 1985 Feb 25;260(4):2295-300.

Cell-specific differences in membrane beta-glucosidase from normal and Gaucher cells.正常细胞和戈谢细胞中膜β-葡萄糖苷酶的细胞特异性差异。

Biochim Biophys Acta. 1977 Feb 9;480(2):442-9. doi: 10.1016/0005-2744(77)90036-5.

Comparison of synthetic and natural glucosylceramides as substrate for glucosylceramidase assay.合成型与天然型葡萄糖神经酰胺作为葡萄糖神经酰胺酶测定底物的比较。

Clin Chim Acta. 1982 Jan 5;118(1):1-7. doi: 10.1016/0009-8981(82)90221-2.

beta-Glucoside hydrolase activity of normal and glucosylceramidotic cultured human skin fibroblasts.正常及葡糖神经酰胺贮积症培养的人皮肤成纤维细胞的β-葡萄糖苷水解酶活性

J Biol Chem. 1977 Feb 10;252(3):825-9.

Multiple forms of human skin fibroblast beta-glucosidase and their activation by fibroblast monosialoglycosphingolipids.

Prog Clin Biol Res. 1982;95:385-403.

Gaucher's disease II. Studies on the kinetics of beta-glucosidase and the effects of sodium taurocholate in normal and Gaucher tissues.戈谢病II型。正常组织和戈谢组织中β-葡萄糖苷酶动力学及牛磺胆酸钠作用的研究。

Pediatr Res. 1980 Jan;14(1):54-9. doi: 10.1203/00006450-198001000-00013.

Gaucher disease. III. Substrate specificity of glucocerebrosidase and the use of nonlabeled natural substrates for the investigation of patients.戈谢病。III. 葡萄糖脑苷脂酶的底物特异性及使用未标记天然底物对患者进行研究

Am J Hum Genet. 1980 Sep;32(5):670-80.

Qualitative characteristics of the beta-glucosidase isozymes.β-葡萄糖苷酶同工酶的定性特征

Prog Clin Biol Res. 1982;95:415-41.

Metabolism of glucosyl [13H]ceramide by human skin fibroblasts from normal and glucosylceramidotic subjects.

J Biol Chem. 1975 May 25;250(10):3966-71.

引用本文的文献

A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C.戈谢病的结构生物学导览：酸性β-葡萄糖苷酶和鞘脂激活蛋白C

Enzyme Res. 2011;2011:973231. doi: 10.4061/2011/973231. Epub 2011 Nov 22.

Mutations of glucocerebrosidase: discrimination of neurologic and non-neurologic phenotypes of Gaucher disease.葡糖脑苷脂酶突变：戈谢病神经型和非神经型表型的鉴别

Proc Natl Acad Sci U S A. 1982 Sep;79(18):5607-10. doi: 10.1073/pnas.79.18.5607.

Evidence for the deficiency of beta-glucosidase-activating factor in fibroblasts of patients with I-cell disease.I型细胞病患者成纤维细胞中β-葡萄糖苷酶激活因子缺乏的证据。

Hum Genet. 1982;62(1):66-9. doi: 10.1007/BF00295605.

Am J Hum Genet. 1980 Sep;32(5):670-80.

Isoenzymes of membrane-bound beta-glucosidase of human spleen.人脾脏膜结合β-葡萄糖苷酶的同工酶

Biochem J. 1980 Feb 1;185(2):541-3. doi: 10.1042/bj1850541.

Characterization of the phospholipid requirement of a rat liver beta-glucosidase.大鼠肝脏β-葡萄糖苷酶磷脂需求的特性研究

Biochem J. 1984 Dec 1;224(2):515-24. doi: 10.1042/bj2240515.

Impaired lysosomes in a temperature-sensitive mutant of Chinese hamster ovary cells.中国仓鼠卵巢细胞温度敏感突变体中溶酶体功能受损。

J Cell Biol. 1989 Jun;108(6):2211-9. doi: 10.1083/jcb.108.6.2211.

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