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Effect of glycine substitutions on alpha 5(IV) chain structure and structure-phenotype correlations in Alport syndrome
Wang, YF; Ding, J; Wang, F; Bu, DF
关键词Collagen Type Iv Nephritis Genotype Hereditary Phenotype Glycine Protein Structure Amino Acid Substitution Secondary Circular Dichroism
刊名BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
2004-04-16
DOI10.1016/j.bbrc.2004.02.168
316期:4页:1143-1149
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Biochemistry & Molecular Biology ; Biophysics
研究领域[WOS]Biochemistry & Molecular Biology ; Biophysics
关键词[WOS]GLOMERULAR-BASEMENT-MEMBRANE ; AMINO-ACID-SEQUENCE ; IV COLLAGEN ; COL4A5 GENE ; IN-VIVO ; MUTATIONS ; PROTEINS ; PEPTIDES ; DOMAIN ; MODEL
英文摘要

The phenotype variety caused by glycine substitutions in alpha5(IV) chain in X-linked Alport syndrome (XLAS) prompted the complexity of structure changes of alpha5(IV) chain that was little to know now. In this study, we expressed a domain of alpha5(IV) chain containing different glycine substitutions (G1015V and G1030S, respectively) which were revealed in two XLAS pedigrees with different phenotype severities and the corresponding domain of a control in Escherichia coli. The recombinant proteins were characterized by immunoblot and mass spectrometry and analyzed the secondary structure by using circular dichroism (CD) spectroscopy. CD analysis showed that the recombinant protein containing G1015V mutation identified in the pedigree of juvenile onset XLAS exhibited 12.9% alpha-helix that was not found in the control recombinant protein. The spectrum of the recombinant protein containing G1030S mutation identified in the pedigree of adult-onset XLAS was slightly different from that of the control, that is, mostly with the random coil and the P-sheet, while without alpha-helix. These results demonstrated that two kinds of glycine substitutions. although in the same domain of a5(IV) chain, displayed the distinctly different secondary structures. The changes of the secondary structure could explain the phenotypic diversities of XLAS, which would be hardly understood solely by analyzing genomic DNA or mRNA of alpha5(IV) chain. (C) 2004 Elsevier Inc. All rights reserved.

语种英语
WOS记录号WOS:000220579300026
引用统计
被引频次:2[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/53242
专题北京大学第一临床医学院_儿科
北京大学临床肿瘤学院_核医学科
作者单位Peking Univ, First Hosp, Dept Pediat, Beijing 100871, Peoples R China
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GB/T 7714
Wang, YF,Ding, J,Wang, F,et al. Effect of glycine substitutions on alpha 5(IV) chain structure and structure-phenotype correlations in Alport syndrome[J]. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS,2004,316(4):1143-1149.
APA Wang, YF,Ding, J,Wang, F,&Bu, DF.(2004).Effect of glycine substitutions on alpha 5(IV) chain structure and structure-phenotype correlations in Alport syndrome.BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS,316(4),1143-1149.
MLA Wang, YF,et al."Effect of glycine substitutions on alpha 5(IV) chain structure and structure-phenotype correlations in Alport syndrome".BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS 316.4(2004):1143-1149.
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