Wnt5a Deficiency Leads to Anomalies in Ureteric Tree Development, Tubular Epithelial Cell Organization and Basement Membrane Integrity Pointing to a Role in Kidney Collecting Duct Patterning

PloS One
Ilkka PietiläSeppo J Vainio

Abstract

The Wnts can be considered as candidates for the Congenital Anomaly of Kidney and Urinary Tract, CAKUT diseases since they take part in the control of kidney organogenesis. Of them Wnt5a is expressed in ureteric bud (UB) and its deficiency leads to duplex collecting system (13/90) uni- or bilateral kidney agenesis (10/90), hypoplasia with altered pattern of ureteric tree organization (42/90) and lobularization defects with partly fused ureter trunks (25/90) unlike in controls. The UB had also notably less tips due to Wnt5a deficiency being at E15.5 306 and at E16.5 765 corresponding to 428 and 1022 in control (p<0.02; p<0.03) respectively. These changes due to Wnt5a knock out associated with anomalies in the ultrastructure of the UB daughter epithelial cells. The basement membrane (BM) was malformed so that the BM thickness increased from 46.3 nm to 71.2 nm (p<0.01) at E16.5 in the Wnt5a knock out when compared to control. Expression of a panel of BM components such as laminin and of type IV collagen was also reduced due to the Wnt5a knock out. The P4ha1 gene that encodes a catalytic subunit of collagen prolyl 4-hydroxylase I (C-P4H-I) in collagen synthesis expression and the overall C-P4H enzyme activity were elevated by aroun...Continue Reading

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Citations

Jun 21, 2016·Cancers·Qi XuSeppo Vainio
Dec 7, 2016·Differentiation; Research in Biological Diversity·Kangsun Yun, Alan O Perantoni
Feb 6, 2020·Developmental Dynamics : an Official Publication of the American Association of Anatomists·Elisabeth A Rutledge, Andrew P McMahon
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Feb 19, 2021·Journal of the American Society of Nephrology : JASN·Miguel VerbitskyAli G Gharavi
Mar 16, 2021·Frontiers in Physiology·Eugenia PapakrivopoulouDavid A Long
Apr 21, 2021·Journal of the American Society of Nephrology : JASN·Sarah N LippSarah Calve
May 26, 2021·Development·Karol KaiserVitezslav Bryja

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Methods Mentioned

BETA
genotyping
PCR
Protein assay
Exome Sequencing
transfection

Software Mentioned

- ASW
GROMOS
Mutation Taster
TASSER
F10
PhyloP
Photoshop
SIFT
GROMACS
Imaris

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