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

  title={Association between congenital defects in papillary outgrowth and functional obstruction in Crim1 mutant mice},
  author={Lorine Wilkinson and Nyoman D. Kurniawan and Yu Leng Phua and Michael J Nguyen and Joan Li and Graham J. Galloway and Hikaru Hashitani and Richard J. Lang and Melissa H. Little},
  journal={The Journal of Pathology},
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. Dynamic magnetic resonance imaging using Magnevist showed fast development of hypo‐intense signal in the kidneys of Crim1$^{KST264/KST264}$ mice, suggesting pooling of filtrate within the renal… 
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The requirement for normal vascular development in collecting duct patterning, development of appropriate nephron architecture, and perinatal papillary maturation, such that disturbances contribute to obstructive nephropathy is highlighted.
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Loss of renal microvascular integrity in postnatal Crim1 hypomorphic transgenic mice.
This study suggests that Crim1 is involved in endothelial maintenance and integrity and its loss contributes to a primary defect in the extraglomerular vasculature.
Crim1KST264/KST264 mice implicate Crim1 in the regulation of vascular endothelial growth factor-A activity during glomerular vascular development.
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Crim1KST264/KST264 mice display a disruption of the Crim1 gene resulting in perinatal lethality with defects in multiple organ systems
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    Developmental dynamics : an official publication of the American Association of Anatomists
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The severe and complex phenotype observed in Crim1KST264/K ST264 mice highlights the importance of Crim1 in numerous aspects of organogenesis.
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