I型碳水化合物缺乏糖蛋白综合征：通过钙连蛋白结合分析确定新合成糖蛋白的寡糖结构 - Suppr

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超能文献

Carbohydrate-deficient glycoprotein syndrome type I: determination of the oligosaccharide structure of newly synthesized glycoproteins by analysis of calnexin binding.

作者信息

Marquardt T, Ullrich K, Niehues R, Koch H G, Harms E

机构信息

Klinik und Poliklinik für Kinderheilkunde, Münster, Germany.

出版信息

J Inherit Metab Dis. 1996;19(2):246-50. doi: 10.1007/BF01799441.

DOI:10.1007/BF01799441

PMID:8739977

Abstract

摘要

相似文献

Carbohydrate-deficient glycoprotein syndrome type I: determination of the oligosaccharide structure of newly synthesized glycoproteins by analysis of calnexin binding.I型碳水化合物缺乏糖蛋白综合征：通过钙连蛋白结合分析确定新合成糖蛋白的寡糖结构

J Inherit Metab Dis. 1996;19(2):246-50. doi: 10.1007/BF01799441.

Role of N-linked oligosaccharide recognition, glucose trimming, and calnexin in glycoprotein folding and quality control.N-连接寡糖识别、葡萄糖修剪和钙连蛋白在糖蛋白折叠及质量控制中的作用。

Proc Natl Acad Sci U S A. 1994 Feb 1;91(3):913-7. doi: 10.1073/pnas.91.3.913.

The molecular chaperone calnexin binds Glc1Man9GlcNAc2 oligosaccharide as an initial step in recognizing unfolded glycoproteins.

J Biol Chem. 1995 Mar 3;270(9):4697-704. doi: 10.1074/jbc.270.9.4697.

Oligosaccharide binding characteristics of the molecular chaperones calnexin and calreticulin.分子伴侣钙连蛋白和钙网蛋白的寡糖结合特性

Biochemistry. 1998 Mar 10;37(10):3480-90. doi: 10.1021/bi972465g.

Carbohydrate-deficient glycoprotein syndrome type 1: correction of the glycosylation defect by deprivation of glucose or supplementation of mannose.1型糖基化缺陷糖蛋白综合征：通过葡萄糖剥夺或甘露糖补充纠正糖基化缺陷

Glycoconj J. 1998 May;15(5):499-505. doi: 10.1023/a:1006939104442.

How N-linked oligosaccharides affect glycoprotein folding in the endoplasmic reticulum.N-连接寡糖如何影响内质网中糖蛋白的折叠。

Mol Biol Cell. 1994 Mar;5(3):253-65. doi: 10.1091/mbc.5.3.253.

[Carbohydrate-deficient glycoprotein syndrome and glycosylation of N-glycoproteins].

Arch Pediatr. 1999;6 Suppl 2:206s-208s. doi: 10.1016/s0929-693x(99)80414-x.

Monoglucosylated oligomannosides are released during the degradation process of newly synthesized glycoproteins.单葡萄糖基化寡甘露糖在新合成糖蛋白的降解过程中被释放出来。

J Biol Chem. 2001 Jun 22;276(25):22307-12. doi: 10.1074/jbc.M101077200. Epub 2001 Apr 6.

Alteration of mannose transport in fibroblasts from type I carbohydrate deficient glycoprotein syndrome patients.

Biochim Biophys Acta. 1999 Mar 30;1453(3):369-77. doi: 10.1016/s0925-4439(99)00009-5.

Calnexin discriminates between protein conformational states and functions as a molecular chaperone in vitro.钙连接蛋白能够区分蛋白质的构象状态，并在体外作为分子伴侣发挥作用。

Mol Cell. 1999 Sep;4(3):331-41. doi: 10.1016/s1097-2765(00)80335-4.

引用本文的文献

A zebrafish model of PMM2-CDG reveals altered neurogenesis and a substrate-accumulation mechanism for N-linked glycosylation deficiency.PMM2-CDG 的斑马鱼模型揭示了神经发生的改变和 N 连接糖基化缺陷的底物积累机制。

Mol Biol Cell. 2012 Nov;23(21):4175-87. doi: 10.1091/mbc.E12-05-0411. Epub 2012 Sep 5.

Analysis of glycosylation in CDG-Ia fibroblasts by fluorophore-assisted carbohydrate electrophoresis: implications for extracellular glucose and intracellular mannose 6-phosphate.通过荧光团辅助碳水化合物电泳分析先天性糖基化障碍I型a型成纤维细胞中的糖基化：对细胞外葡萄糖和细胞内甘露糖6-磷酸的影响

J Biol Chem. 2005 May 6;280(18):17901-9. doi: 10.1074/jbc.M500510200. Epub 2005 Feb 11.

本文引用的文献

The endoplasmic reticulum as a protein-folding compartment.作为蛋白质折叠区室的内质网。

Trends Cell Biol. 1992 Aug;2(8):227-31. doi: 10.1016/0962-8924(92)90309-b.

Membrane glycoprotein folding, oligomerization and intracellular transport: effects of dithiothreitol in living cells.膜糖蛋白折叠、寡聚化及细胞内运输：二硫苏糖醇对活细胞的影响

EMBO J. 1993 May;12(5):2151-7. doi: 10.1002/j.1460-2075.1993.tb05863.x.

Sugar chains of serum transferrin from patients with carbohydrate deficient glycoprotein syndrome. Evidence of asparagine-N-linked oligosaccharide transfer deficiency.碳水化合物缺乏糖蛋白综合征患者血清转铁蛋白的糖链。天冬酰胺 - N - 连接寡糖转移缺陷的证据。

J Biol Chem. 1993 Mar 15;268(8):5783-9.

The carbohydrate-deficient glycoprotein syndromes: an overview.碳水化合物缺乏糖蛋白综合征概述

J Inherit Metab Dis. 1993;16(5):813-20. doi: 10.1007/BF00714272.

Electrospray ionization-mass spectrometric analysis of serum transferrin isoforms in patients with carbohydrate-deficient glycoprotein syndrome.碳水化合物缺乏糖蛋白综合征患者血清转铁蛋白异构体的电喷雾电离-质谱分析

J Biochem. 1993 Dec;114(6):766-9. doi: 10.1093/oxfordjournals.jbchem.a124253.

Carbohydrate-deficient glycoprotein syndrome: not an N-linked oligosaccharide processing defect, but an abnormality in lipid-linked oligosaccharide biosynthesis?碳水化合物缺乏糖蛋白综合征：并非N-连接寡糖加工缺陷，而是脂连接寡糖生物合成异常？

J Clin Invest. 1994 Nov;94(5):1901-9. doi: 10.1172/JCI117540.

Normal N-oligosaccharyltransferase activity in fibroblasts from patients with carbohydrate-deficient glycoprotein syndrome.碳水化合物缺乏糖蛋白综合征患者成纤维细胞中的正常N-寡糖基转移酶活性。

J Inherit Metab Dis. 1994;17(5):541-4. doi: 10.1007/BF00711588.

Carbohydrate-deficient glycoprotein syndrome (CDGS)--glycosylation, folding and intracellular transport of newly synthesized glycoproteins.

Eur J Cell Biol. 1995 Mar;66(3):268-73.

Glucose trimming and reglucosylation determine glycoprotein association with calnexin in the endoplasmic reticulum.葡萄糖修剪和再糖基化决定了内质网中糖蛋白与钙连蛋白的结合。

Cell. 1995 May 5;81(3):425-33. doi: 10.1016/0092-8674(95)90395-x.

Enzymes involved in the synthesis of mannose-6-phosphate from glucose are normal in carbohydrate deficient glycoprotein syndrome fibroblasts.在碳水化合物缺乏糖蛋白综合征成纤维细胞中，参与从葡萄糖合成6-磷酸甘露糖的酶是正常的。

Biochem Biophys Res Commun. 1995 Mar 17;208(2):517-22. doi: 10.1006/bbrc.1995.1369.

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