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Magnetic resonance microscopy of renal and biliary abnormalities in excised
tissues from a mouse model of autosomal recessive polycystic kidney disease
#MMPMID26320214
Lee CH
; O'Connor AK
; Yang C
; Tate JM
; Schoeb TR
; Flint JJ
; Blackband SJ
; Guay-Woodford LM
Physiol Rep
2015[Aug]; 3
(8
): ? PMID26320214
show ga
Polycystic kidney disease (PKD) is transmitted as either an autosomal dominant or
recessive trait and is a major cause of renal failure and liver fibrosis. The cpk
mouse model of autosomal recessive PKD (ARPKD) has been extensively characterized
using standard histopathological techniques after euthanasia. In the current
study, we sought to validate magnetic resonance microscopy (MRM) as a robust tool
for assessing the ARPKD phenotype. We used MRM to evaluate the liver and kidney
of wild-type and cpk animals at resolutions <100 ?m and generated
three-dimensional (3D) renderings for pathological evaluation. Our study
demonstrates that MRM is an excellent method for evaluating the complex, 3D
structural defects in this ARPKD mouse model. We found that MRM was equivalent to
water displacement in assessing kidney volume. Additionally, using MRM we
demonstrated for the first time that the cpk liver exhibits less extensive ductal
arborization, that it was reduced in volume, and that the ductal volume was
disproportionately smaller. Histopathology indicates that this is a consequence
of bile duct malformation. With its reduced processing time, volumetric
information, and 3D capabilities, MRM will be a useful tool for future in vivo
and longitudinal studies of disease progression in ARPKD. In addition, MRM will
provide a unique tool to determine whether the human disease shares the newly
appreciated features of the murine biliary phenotype.
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