Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10833981 | Molecular Genetics and Metabolism | 2011 | 10 Pages |
Abstract
⺠Homotrimeric type I collagen is the pathogenic in Col1A2-deficient glomerulopathy. ⺠Homotrimeric type I collagen is resistant to MMP degradation. ⺠Homotrimeric type I collagen accumulation in glomeruli due to MMP resistance.
Keywords
ECMHBSSPMSFOIMPSRUPRTRS+/−PBSDAB3,3′ diaminobenzidine tetrahydrochlorideMMPHprt+/+−/−BSAHanks balanced salt solutionpicrosirius redbovine serum albuminGlomerular sclerosisepithelial-to-mesenchymal-transitionIHCImmunohistochemistryOsteogenesis imperfectaEMTendoplasmic reticulumPhosphate buffered salinephenylmethyl sulfonyl fluorideFibrosisExtracellular matrixmatrix metalloproteinasehypoxanthine-guanine phosphoribosyltransferaseUnfolded protein responseCollagen
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Authors
Anna M. Roberts-Pilgrim, Elena Makareeva, Matthew H. Myles, Cynthia L. Besch-Williford, Amanda C. Brodeur, Andrew L. Walker, Sergey Leikin, Craig L. Franklin, Charlotte L. Phillips,