Journal article
Distinct sites of renal fibrosis in Crim1 mutant mice arise from multiple cellular origins
YL Phua, N Martel, DJ Pennisi, MH Little, L Wilkinson
Journal of Pathology | Published : 2013
DOI: 10.1002/path.4155
Abstract
Crim1 is a transmembrane protein that regulates the bioavailability of growth factors such as VEGFA. Crim1KST264/ KST264 hypomorphic mice develop renal disease characterized by glomerular cysts and loss of endothelial integrity, progressing to peritubular and pericystic fibrosis. Peritubular capillary endothelial cells display morphological changes as well as detachment from the basement membrane. In this study, gene expression profiling of CD31+ endothelial cells isolated from Crim1KST264/KST264 kidneys showed up-regulation of transcripts associated with fibrosis (Col3a1, Loxl1), endothelial dysfunction (Abp1, Dcn, Lcn2), biomarkers of renal damage (Lcn2, Havcr1/Kim1) as well as evidence fo..
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Awarded by NHMRC
Funding Acknowledgements
We acknowledge the support staff of the University of Queensland Biological Resources Animal Facility; the SRC Microarray facility; the QFAB Facility for Ingenuity Pathway Analysis access; the ACRF/IMB Cancer Biology Imaging Facility for the confocal microscopes; the University of Queensland Centre for Microscopy and Microanalysis; the laboratory of Peter Koopman for the Mini MACS equipment; and Milena Gongora and Joan Li for technical assistance. YLP holds an UQIRTA and UQRS scholarship. MHL is an NHMRC Principal Research Fellow (Grant No. 511032).