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学科主题: 基础医学
题名:
A two-dimensional protein fragmentation-proteomic study of neuronal ceroid lipofuscinoses: Identification and characterization of differentially expressed proteins
作者: Wang, Peirong2,3; Ju, Weina1; Wu, Dan2,3; Wang, Li2,3; Yan, Ming2,3; Zou, Junhua2,3; He, Bing2,3; Jenkins, Edmund C.1; Brown, W. Ted1; Zhong, Nanbert1,2,3
关键词: Neuronal ceroid lipofuscinosis ; Batten disease ; Proteomics ; PF2D ; Cytoskeleton
刊名: JOURNAL OF CHROMATOGRAPHY B-ANALYTICAL TECHNOLOGIES IN THE BIOMEDICAL AND LIFE SCIENCES
发表日期: 2011-02-15
DOI: 10.1016/j.jchromb.2010.12.011
卷: 879, 期:5-6, 页:304-316
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Biochemical Research Methods ; Chemistry, Analytical
研究领域[WOS]: Biochemistry & Molecular Biology ; Chemistry
关键词[WOS]: BATTEN-DISEASE ; ANNEXIN-I ; NUCLEAR-LOCALIZATION ; ALZHEIMERS-DISEASE ; OXIDATIVE STRESS ; GENE-EXPRESSION ; MESSENGER-RNA ; MOUSE MODEL ; CATHEPSIN-D ; CELLS
英文摘要:

The neuronal ceroid lipofuscinoses (NCLs) are a group of neuronal degenerative diseases that primarily affect children. Previously we hypothesized that the similarity of the phenotypes among the variant subtypes of NCL suggests that the NCLs share a common metabolic functional pathway. To test our hypothesis, we have studied several candidate proteins identified using a proteomic approach. We analyzed their differential expression and cataloged their functions and involved pathways. Forty protein peaks, differentially expressed in NCLs, were selected from two-dimensional protein fragmentation (PF2D) maps and twenty-four proteins were identified by MALDI-TOF-MS or LC-ESI-MS/MS. Six proteins were verified by further Western blotting. Our results showed that annexin A1, annexin A2, and vimentin were significantly down-regulated in NCL1, NCL2, NCL3, and NCL8 cells; galectin-1 was down-regulated in NCL1, NCL3, and NCL8 but up-regulated in NCL2 cells; and isoform 5 of caldesmon was up-regulated in all NCL cell types. The histone 2B was down-regulated in NCL3. Functional analysis showed that the differentially expressed proteins identified by PF2D could be grouped into categories of intermediate filaments, cell motility, apoptosis, cytoskeleton, membrane trafficking, calcium binding, nucleosome assembly, pigment granule and cell development. Immunocytochemistry revealed nuclear translocalization of annexin A1 in CLN2-deficient fibroblasts and abnormal distribution of L-caldesmon in cultured CLN1, CLN2, CLN3 and CLN8-deficient fibroblasts. Finding differentially expressed proteins in variant NCLs, which showed disturbances of cytoskeleton, RAGE-dependent cellular pathways and decreased glycolysis provides evidence supporting our hypothesis. These findings may contribute to the discovery of molecular biomarkers and may help further elucidate the pathogenic mechanisms underlying the NCLs. (C) 2010 Elsevier B.V. All rights reserved.

语种: 英语
所属项目编号: 985-2-035-39 ; 2007CB511902 ; 30671157
项目资助者: Chinese Ministry of Education ; Ministry of Science and Technology ; National Nature Science Foundation ; New York State Office of People with Developmental Disabilities [NYS OPWDD]
WOS记录号: WOS:000287564800002
Citation statistics:
内容类型: 期刊论文
版本: 出版稿
URI标识: http://ir.bjmu.edu.cn/handle/400002259/50087
Appears in Collections:基础医学院_医学遗传学系_期刊论文

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作者单位: 1.New York State Inst Basic Res Dev Disabil, Dept Human Genet, Staten Isl, NY 10314 USA
2.Peking Univ, Hlth Sci Ctr, Dept Med Genet, Beijing 100871, Peoples R China
3.Peking Univ, Ctr Med Genet, Beijing 100871, Peoples R China

Recommended Citation:
Wang, Peirong,Ju, Weina,Wu, Dan,et al. A two-dimensional protein fragmentation-proteomic study of neuronal ceroid lipofuscinoses: Identification and characterization of differentially expressed proteins[J]. JOURNAL OF CHROMATOGRAPHY B-ANALYTICAL TECHNOLOGIES IN THE BIOMEDICAL AND LIFE SCIENCES,2011,879(5-6):304-316.
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