کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
10833209 | 1065789 | 2013 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Pathogenesis of mitral valve disease in mucopolysaccharidosis VII dogs
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کلمات کلیدی
HGFGUSBGAGECMNIHCCLDTTTLR4OSMIDUAα-L-iduronidaseHSCTSLRPCTSRLUOMIMChordae tendineaechemokine (C–C motif) ligandMMPTNFZ-Phe-Arg-AMCβ-glucuronidase - β-گلوکورونیدازenzyme replacement therapy - آنزیم جایگزین درمانEDTA - اتیلن دی آمین تترا استیک اسید Ethylenediaminetetraacetic acid - اتیلینیدامین تتراستیک اسیدoncostatin M - اوکتواستاتین مinterleukin - اینترلوکینRetroviral vector - بردار RetroviralLysosomal storage disease - بیماری ذخیره سازی لیزوزومیtumor necrosis factor-α - تومور نکروز عامل αIntravenous - داخل وریدیmitral valve - دریچه میترالdithiothreitol - دیتیوتریتولDog - سگHepatocyte growth factor - عامل رشد هپاتوسیتNational Institutes of Health - مؤسسات ملی بهداشتExtracellular matrix - ماتریکس خارج سلولیmatrix metalloproteinase - ماتریکس متالوپروتئینازreverse-transcription - معکوس رونویسیMucopolysaccharidosis - موکوپلیساکاریدوزElectron microscopy - میکروسکوپ الکترونیmitral regurgitation - نارسایی میترالMPs - نمایندگان مجلسERT - هستندOnline Mendelian Inheritance in Man - وراث آنلاین مندلیان در انسانsmall leucine-rich proteoglycan - پروتئگلیکان غنی از لوسین استHematopoietic stem cell transplantation - پیوند مغز استخوانCathepsin - کاتپسینCollagen - کلاژنGlycosaminoglycan - گلیکوزآمینوگلیکانToll-like receptor 4 - گیرنده تله مانند 4
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Mucopolysaccharidosis VII (MPS VII) is due to the deficient activity of β-glucuronidase (GUSB) and results in the accumulation of glycosaminoglycans (GAGs) in lysosomes and multisystemic disease with cardiovascular manifestations. The goal here was to determine the pathogenesis of mitral valve (MV) disease in MPS VII dogs. Untreated MPS VII dogs had a marked reduction in the histochemical signal for structurally-intact collagen in the MV at 6 months of age, when mitral regurgitation had developed. Electron microscopy demonstrated that collagen fibrils were of normal diameter, but failed to align into large parallel arrays. mRNA analysis demonstrated a modest reduction in the expression of genes that encode collagen or collagen-associated proteins such as the proteoglycan decorin which helps collagen fibrils assemble, and a marked increase for genes that encode proteases such as cathepsins. Indeed, enzyme activity for cathepsin B (CtsB) was 19-fold normal. MPS VII dogs that received neonatal intravenous injection of a gamma retroviral vector had an improved signal for structurally-intact collagen, and reduced CtsB activity relative to that seen in untreated MPS VII dogs. We conclude that MR in untreated MPS VII dogs was likely due to abnormalities in MV collagen structure. This could be due to upregulation of enzymes that degrade collagen or collagen-associated proteins, to the accumulation of GAGs that compete with proteoglycans such as decorin for binding to collagen, or to other causes. Further delineation of the etiology of abnormal collagen structure may lead to treatments that improve biomechanical properties of the MV and other tissues.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Molecular Genetics and Metabolism - Volume 110, Issue 3, November 2013, Pages 319-328
Journal: Molecular Genetics and Metabolism - Volume 110, Issue 3, November 2013, Pages 319-328
نویسندگان
Paul W. Bigg, Guilherme Baldo, Meg M. Sleeper, Patricia A. O'Donnell, Hanqing Bai, Venkata R.P. Rokkam, Yuli Liu, Susan Wu, Roberto Giugliani, Margret L. Casal, Mark E. Haskins, Katherine P. Ponder,