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学科主题: 临床医学
题名:
Effect of glycine substitutions on alpha 5(IV) chain structure and structure-phenotype correlations in Alport syndrome
作者: Wang, YF; Ding, J; Wang, F; Bu, DF
关键词: nephritis, hereditary ; collagen type IV ; genotype ; phenotype ; protein structure, secondary ; glycine ; amino acid substitution ; circular dichroism
刊名: BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
发表日期: 2004-04-16
DOI: 10.1016/j.bbrc.2004.02.168
卷: 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
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/53242
Appears in Collections:北京大学第一临床医学院_儿科_期刊论文

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作者单位: Peking Univ, First Hosp, Dept Pediat, Beijing 100871, Peoples R China

Recommended Citation:
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.
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