Loss of the BMP antagonist USAG-1 ameliorates disease in a mouse model of the progressive hereditary kidney disease Alport syndrome

The glomerular basement membrane (GBM) is a key component of the filtering unit in the kidney. Mutations involving any of the collagen IV genes (COL4A3, COL4A4, and COL4A5) affect GBM assembly and cause Alport syndrome, a progressive hereditary kidney disease with no definitive therapy. Previously,...

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Veröffentlicht in:	The Journal of clinical investigation 2010-03, Vol.120 (3), p.768-777
Hauptverfasser:	Tanaka, Mari, Asada, Misako, Higashi, Atsuko Y, Nakamura, Jin, Oguchi, Akiko, Tomita, Mayumi, Yamada, Sachiko, Asada, Nariaki, Takase, Masayuki, Okuda, Tomohiko, Kawachi, Hiroshi, Economides, Aris N, Robertson, Elizabeth, Takahashi, Satoru, Sakurai, Takeshi, Goldschmeding, Roel, Muso, Eri, Fukatsu, Atsushi, Kita, Toru, Yanagita, Motoko
Format:	Artikel
Sprache:	eng
Schlagworte:	Ablation Acute Kidney Injury - genetics Acute Kidney Injury - metabolism Acute Kidney Injury - pathology Age Alport's syndrome Animal models in research Animals Biomedical research Bone Morphogenetic Protein 7 - antagonists & inhibitors Bone Morphogenetic Protein 7 - genetics Bone Morphogenetic Protein 7 - metabolism Bone morphogenetic proteins Bone Morphogenetic Proteins - genetics Bone Morphogenetic Proteins - metabolism Collagen Collagen Type IV - genetics Collagen Type IV - metabolism Creatinine Disease Models, Animal Genes Genetic aspects Glomerular Basement Membrane - metabolism Glomerular Basement Membrane - pathology Glomerular Mesangium - metabolism Glomerular Mesangium - pathology Humans Kidney diseases Kidney Tubules - metabolism Kidney Tubules - pathology Mice Mice, Knockout Mutation Nephritis, Hereditary - genetics Nephritis, Hereditary - metabolism Nephritis, Hereditary - pathology Physiological aspects Proteins Risk factors
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