Evidence for activation of the unfolded protein response in collagen IV nephropathies

Thin-basement-membrane nephropathy (TBMN) and Alport syndrome (AS) are progressive collagen IV nephropathies caused by mutations in COL4A3/A4/A5 genes. These nephropathies invariably present with microscopic hematuria and frequently progress to proteinuria and CKD or ESRD during long-term follow-up....

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Vydáno v:	Journal of the American Society of Nephrology Ročník 25; číslo 2; s. 260
Hlavní autoři:	Pieri, Myrtani, Stefanou, Charalambos, Zaravinos, Apostolos, Erguler, Kamil, Stylianou, Kostas, Lapathitis, George, Karaiskos, Christos, Savva, Isavella, Paraskeva, Revekka, Dweep, Harsh, Sticht, Carsten, Anastasiadou, Natassa, Zouvani, Ioanna, Goumenos, Demetris, Felekkis, Kyriakos, Saleem, Moin, Voskarides, Konstantinos, Gretz, Norbert, Deltas, Constantinos
Médium:	Journal Article
Jazyk:	angličtina
Vydáno:	United States 01.02.2014
Témata:	Animals Autoantigens - genetics Autoantigens - physiology Biopsy Cells, Cultured Collagen Type IV - deficiency Collagen Type IV - genetics Collagen Type IV - physiology Disease Models, Animal DNA-Binding Proteins - metabolism Endoplasmic Reticulum Stress - physiology Gene Expression Profiling Gene Knock-In Techniques Glomerular Basement Membrane - metabolism Glomerular Basement Membrane - pathology Heat-Shock Proteins - biosynthesis Heat-Shock Proteins - genetics Heterozygote Humans Kidney - metabolism Kidney - pathology Mice Mutation, Missense Nephritis, Hereditary - genetics Nephritis, Hereditary - metabolism Nephritis, Hereditary - pathology Oligonucleotide Array Sequence Analysis Podocytes - metabolism Podocytes - pathology Point Mutation Protein Array Analysis Protein Transport Recombinant Fusion Proteins Regulatory Factor X Transcription Factors RNA Interference RNA, Small Interfering - pharmacology Transcription Factors - metabolism Transfection Unfolded Protein Response - physiology
ISSN:	1533-3450, 1533-3450
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