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available</cell_type><repository>GPMDB</repository><pubmed_abstract>Fuchs' endothelial corneal dystrophy (FECD) is a major corneal disorder affecting the innermost part of the cornea, leading to visual impairment. As the morphological changes in FECD are mainly observed in the extracellular matrix of the Descemet's membrane/endothelial layer, we determined the protein profiles of diseased and control tissues using two relative quantitation MS methods. The first quantitation method, based on the areas of the extracted ion chromatograms, quantified the 51 and 48 most abundant proteins of the Descemet's membrane/endothelial layer in patient and control tissues, respectively, of which 10 were significantly regulated. The results indicated that the level of type VIII collagen was unaltered even though the protein previously has been shown to be implicated in familial early-onset forms of the disease. Using the second relative quantitation method, iTRAQ, we identified 22 differentially regulated proteins, many of which are extracellular proteins known to be involved in proper assembly of the basement membrane in other tissues. In total, 26 differentially regulated proteins were identified, of which 6 proteins were regulated in both methods. These results support that the morphological changes observed in FECD are caused in part by an aberrant assembly of the extracellular matrix within the Descemet's membrane/endothelial layer.</pubmed_abstract><pubmed_title>Proteomics of Fuchs' endothelial corneal dystrophy support that the extracellular matrix of Descemet's membrane is disordered.</pubmed_title><pubmed_authors>Poulsen Ebbe Toftgaard ET,Dyrlund Thomas F TF,Runager Kasper K,Scavenius Carsten C,Krogager Toke Peter TP,Højrup Peter P,Thøgersen Ida B IB,Sanggaard Kristian W KW,Vorum Henrik H,Hjortdal Jesper J,Enghild Jan J JJ,</pubmed_authors><pubmed_authors>Poulsen Ebbe Toftgaard ET, Dyrlund Thomas F TF, Runager Kasper K, Scavenius Carsten C, Krogager Toke Peter TP, Højrup Peter P, Thøgersen Ida B IB, Sanggaard Kristian W KW, Vorum Henrik H, Hjortdal Jesper J, Enghild Jan J JJ</pubmed_authors><name_synonyms>"corneal dystrophy" EXACT [CSP2005:1254-7727], Matrix, membrane, membrane of organ, Extracellular Matrices, "Corneal dystrophy (disorder)" EXACT [SNOMEDCT_2005_07_31:5587004], Matrices, "Corneal Dystrophy" EXACT [NCI2004_11_17:C34513], Extracellular, proteinaceous extracellular matrix, "corneal dystrophy" EXACT [CSP2005:1114-8656], membranous organ component.</name_synonyms><description_synonyms>cornea, advanced, Procedures, Slf, C8H10N2S, tunica cornea, Somatomedin-C, Matrix, Gene, FPH2, Ethylisothiamide, preT.ETP.Th, Matrices, Endoepithelial corneal dystrophy, cornea of camera-type eye, "corneal dystrophy" EXACT [CSP2005:1254-7727], method, Method, Entire cornea, "Corneal Dystrophy" EXACT [NCI2004_11_17:C34513], method used in an experiment, Etionamid, Studies, Gene Products, Extracellular Matrices, 2-ethylpyridine-4-carbothioamide, FECD, corneas, human disease, Ethionamide, reference sample, ethionamidum, layer, Etioniamid, Tissue, somatomedin-C, SF, Ethioniamide, Kitl, cell sheath, proteins, procedures, free, Mast cell growth factor, Study, Methodological Studies, IGF1, Extracellular, Mechano growth factor, Trecator, Homo sapiens disease, Soluble KIT ligand, ETH, Sl, Controlled, ETP, IGF-I, membrane, data, Controlling, steel factor, hematopoietic growth factor KL, Proteins, lamina, Ethyonomide, somatomedin, Ethinamide, membranous organ component, Procedure, results, sKITLG, polypeptide, Experiment, "Corneal dystrophy (disorder)" EXACT [SNOMEDCT_2005_07_31:5587004], Corneas, Diseases., mechano growth factor, Protein, precocious, techniques, Igf-1, "corneal dystrophy" EXACT [CSP2005:1114-8656], SHEP7, membrane of organ, mast cell growth factor, etionamida, Stem cell factor, patient, Methodological, STAT5, Methodological Study, early, plan specification, Protein Gene Products, Gene Proteins, stem cell factor, KL-1, MGF, c-Kit ligand, sheath of cells, layer of cells, KITLG, SCF, Etionamide, Proteomes, proteinaceous extracellular matrix, methodology, Late hereditary endothelial dystrophy</description_synonyms><pubmed_title_synonyms>"corneal dystrophy" EXACT [CSP2005:1254-7727], posterior limiting membrane, posterior limiting lamina of cornea., lamina limitans posterior corneae, "Corneal dystrophy (disorder)" EXACT [SNOMEDCT_2005_07_31:5587004], "Corneal Dystrophy" EXACT [NCI2004_11_17:C34513], Extracellular, Descemet's posterior elastic lamina, Matrix, lamina limitans posterior, posterior limiting lamina, Extracellular Matrices, Matrices, proteinaceous extracellular matrix, "corneal dystrophy" EXACT [CSP2005:1114-8656], Descemet membrane</pubmed_title_synonyms><pubmed_abstract_synonyms>basement membrane, atypia, Controlling, cornea, lamina limitans posterior corneae, advanced, lamina densa, Basement, Procedures, Basement Laminas, aberrant, membrana basalis, Basement Lamina, tunica cornea, Proteins, basement membrane of connective tissue, lamina, Matrix, Gene, posterior limiting lamina, defective, Procedure, Matrices, Endoepithelial corneal dystrophy, results, cornea of camera-type eye, Laminas, "corneal dystrophy" EXACT [CSP2005:1254-7727], polypeptide, method, "Corneal dystrophy (disorder)" EXACT [SNOMEDCT_2005_07_31:5587004], sheath of cells., Corneas, Method, Entire cornea, "Corneal Dystrophy" EXACT [NCI2004_11_17:C34513], Descemet's posterior elastic lamina, method used in an experiment, Protein, Diseases, Studies, Gene Products, precocious, Basal Laminas, basement lamina, atypical, techniques, Extracellular Matrices, Basement Membranes, "corneal dystrophy" EXACT [CSP2005:1114-8656], FECD, posterior limiting membrane, Membranes, corneas, human disease, reference sample, posterior limiting lamina of cornea, layer, Basal, Tissue, lamina limitans posterior, cell sheath, proteins, patient, Methodological, procedures, Membrane, Methodological Study, Descemet membrane, early, Lamina, plan specification, Protein Gene Products, Study, Gene Proteins, extracellular, Basal Lamina, Methodological Studies, Extracellular, sheath of cells, layer of cells, Homo sapiens disease, s, basal lamina, proteinaceous extracellular matrix, Controlled, 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archive~1</search_domains><search_domains>varsite~0</search_domains><reanalysis_count>0</reanalysis_count><submitter_keywords>Resource Reanalysis</submitter_keywords><citation_count_scaled>0.0</citation_count_scaled><reanalysis_count_scaled>0.0</reanalysis_count_scaled><view_count_scaled>0.019123997532387416</view_count_scaled><download_count_scaled>0.0</download_count_scaled><normalized_connections>1.0</normalized_connections></additional><is_claimable>false</is_claimable><name>Proteomics of Fuchs- Endothelial Corneal Dystrophy support that the extracellular matrix of Descemet-s membrane is disordered.</name><description>Data from ProteomeXchange, PXD ID: PXD000746. Experiment: label-free, file: 2012-10-05 - ETP - Fuchs - Control - 3-2.mgf. Published as part of J Proteome Res. 2014 May 21  . From the Abstract: {{i}} Fuchs- endothelial corneal dystrophy (FECD) is a major corneal disorder affecting the innermost part of the cornea, leading to visual impairment. As the morphological changes in FECD are mainly observed in the extracellular matrix of the Descemet-s membrane/endothelial layer we determined the protein profiles of diseased and control tissues using two relative quantitation MS methods. The first quantitation method based on the areas of the extracted ion chromatograms, quantified the 51 and 48 most abundant proteins of the Descemet-s membrane/endothelial layer in patient and control tissues, respectively, of which 10 were significantly regulated. The results indicated that the level of type VIII collagen was unaltered even though the protein previously has been implicated in familial early onset forms of the disease. {{/i}}</description><dates><submission>2014-05-28</submission></dates><accession>GPM32310000394</accession><cross_references><pubmed>24846694</pubmed><Pride>PXD000746</Pride><Pride Archive>PXD000746</Pride Archive></cross_references></HashMap>