ISOLATION OF THE SERUM FACTOR RESPONSIBLE FOR PSEUDOXANTHOMA ELASTICUM
ISOLATION OF THE SERUM FACTOR RESPONSIBLE FOR PSEUDOXANTHOMA ELASTICUM
批准号:
7959637
负责人:
OLIVIER LE SAUX
金额:
$1.49万
依托单位国家:
美国
项目类别:
财政年份:
2009
资助国家:
美国
项目状态:
已结题
起止时间:
2009-06-01 至 2010-05-31
关键词:
Animal ModelAortaBiological AssayCardiovascular systemCell LineCenters of Research ExcellenceCharacteristicsComputer Retrieval of Information on Scientific Projects DatabaseCulture MediaDefectDepositionDisease modelElastic FiberElastinElectron MicroscopyEventFBN1FiberFibroblastsFractionationFundingGoalsGrantHomeostasisHumanIn VitroIndividualInstitutionLOX geneMass Spectrum AnalysisMolecularNaturePatientsPeptide HydrolasesProductionProteinsProtocols documentationPseudoxanthoma ElasticumResearchResearch PersonnelResourcesSerumSkinSmooth Muscle MyocytesSourceStagingTestingUnited States National Institutes of Healthbasecalcificationfibulin-4in vivostructural biology
中文摘要
这个子项目是众多研究子项目之一
英文摘要
This subproject is one of many research subprojects utilizing the
resources provided by a Center grant funded by NIH/NCRR. The subproject and
investigator (PI) may have received primary funding from another NIH source,
and thus could be represented in other CRISP entries. The institution listed is
for the Center, which is not necessarily the institution for the investigator.
Objectives. The long-term goal of this study is to better understand the remarkable biology and structural organization of elastic fibers. This general goal is best achieved by studying these fibers in vitro and in vivo using both human and/or animal models. The specific goal of our project is to identify and characterize the molecular events leading to elastic fiber alterations and calcification in pseudoxanthoma elasticum (PXE) as a pertinent disease model that should help elucidate the dynamics governing the homeostasis of elastic fiber proteins.
Specific Aim 1: Determine the defect in the assembly of elastic fiber caused by PXE serum in vitro.
We will use primary cell lines of skin fibroblasts and normal aorta smooth muscle cells with culture medium supplemented with serum from PXE patients or normal individuals. The principal elastic fiber components (elastin, fibulin-4, -5, fibrillin-1, -2, MAGP1, LOX and LOXL) will be examined by immunohistology and quantified during various stages of elastic fiber deposition, from pre- to post-confluence. Structural alterations will be evaluated by electron microscopy. The production of elastic fiber-specific proteases by fibroblasts and smooth muscle cells in the presence of PXE serum will also be evaluated by zymography.
Specific Aim 2: Isolate serum factor(s) interfering with elastic fiber formation.
The nature of the PXE serum factor(s) is unknown. We propose to determine the molecular characteristics of the PXE serum active component(s) and establish an isolation protocol based on chromatographic fractionations followed by mass spectroscopy analysis. The fractions will be tested using in vitro functional assays.
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依托单位:
ISOLATION OF THE SERUM FACTOR RESPONSIBLE FOR PSEUDOXANTHOMA ELASTICUM
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项目类别:
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财政年份:--
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依托单位:
海外基金