Association between congenital defects in papillary outgrowth and functional obstruction in Crim1 mutant mice

• Published in: The Journal of pathology Vol. 227; no. 4; pp. 499 - 510
• Main Authors: Wilkinson, Lorine, Kurniawan, Nyoman D, Phua, Yu Leng, Nguyen, Michael J, Li, Joan, Galloway, Graham J, Hashitani, Hikaru, Lang, Richard J, Little, Melissa H
• Format: Journal Article
• Language: English
• Published: Chichester, UK John Wiley & Sons, Ltd 01.08.2012; Wiley
• Subjects: Animals; Biological and medical sciences; Bone Morphogenetic Protein Receptors - genetics; Bone Morphogenetic Protein Receptors - metabolism; Crim1; Dextrans - metabolism; functional obstruction; hydronephrosis; Hydronephrosis - congenital; Hydronephrosis - metabolism; Hydronephrosis - physiopathology; Investigative techniques, diagnostic techniques (general aspects); Kidney Diseases - congenital; Kidney Diseases - metabolism; Kidney Diseases - physiopathology; Kidney Medulla - abnormalities; Kidney Medulla - metabolism; Kidney Medulla - physiopathology; Kidneys; Loop of Henle - metabolism; Magnetic Resonance Imaging; Medical sciences; Mice; Mice, Transgenic; Models, Animal; Muscle Contraction - physiology; Mutation - genetics; Myocytes, Smooth Muscle - pathology; Nephrology. Urinary tract diseases; obstructive nephropathy; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; pyeloureteric peristalsis; Rhodamines - metabolism; Urinary system involvement in other diseases. Miscellaneous; Urinary tract. Prostate gland; Kidney disease; magnetic resonance imaging; Urinary system disease; Rodentia; Congenital anomaly; Urinary tract disease; Nuclear magnetic resonance imaging; Obstruction; Hydronephrosis; Vertebrata; Anatomic pathology; Mammalia; Association; Nephropathy; Mouse; Animal; functional obstruction; Mutation; Crim1; obstructive nephropathy; pyeloureteric peristalsis

Crim1 hypomorphic (Crim1$^{KST264/KST264}$) mice display progressive renal disease characterized by glomerular defects, leaky peritubular vasculature, and progressive interstitial fibrosis. Here we show that 27% of these mice also present with hydronephrosis, suggesting obstructive nephropathy. Dyna...