In:
PLOS ONE, Public Library of Science (PLoS), Vol. 18, No. 5 ( 2023-5-9), p. e0285418-
Kurzfassung:
Fibrillin-1 is a pivotal structural component of the kidney’s glomerulus and peritubular tissue. Mutations in the fibrillin-1 gene result in Marfan syndrome (MFS), an autosomal dominant disease of the connective tissue. Although the kidney is not considered a classically affected organ in MFS, several case reports describe glomerular disease in patients. Therefore, this study aimed to characterize the kidney in the mgΔ lpn -mouse model of MFS. Affected animals presented a significant reduction of glomerulus, glomerulus-capillary, and urinary space, and a significant reduction of fibrillin-1 and fibronectin in the glomerulus. Transmission electron microscopy and 3D-ultrastructure analysis revealed decreased amounts of microfibrils which also appeared fragmented in the MFS mice. Increased collagen fibers types I and III, MMP-9, and α-actin were also observed in affected animals, suggesting a tissue-remodeling process in the kidney. Video microscopy analysis showed an increase of microvessel distribution coupled with reduction of blood-flow velocity, while ultrasound flow analysis revealed significantly lower blood flow in the kidney artery and vein of the MFS mice. The structural and hemodynamic changes of the kidney indicate the presence of kidney remodeling and vascular resistance in this MFS model. Both processes are associated with hypertension which is expected to worsen the cardiovascular phenotype in MFS.
Materialart:
Online-Ressource
ISSN:
1932-6203
DOI:
10.1371/journal.pone.0285418
DOI:
10.1371/journal.pone.0285418.g001
DOI:
10.1371/journal.pone.0285418.g002
DOI:
10.1371/journal.pone.0285418.g003
DOI:
10.1371/journal.pone.0285418.g004
DOI:
10.1371/journal.pone.0285418.g005
DOI:
10.1371/journal.pone.0285418.g006
DOI:
10.1371/journal.pone.0285418.g007
DOI:
10.1371/journal.pone.0285418.g008
DOI:
10.1371/journal.pone.0285418.s001
DOI:
10.1371/journal.pone.0285418.s002
DOI:
10.1371/journal.pone.0285418.s003
DOI:
10.1371/journal.pone.0285418.s004
DOI:
10.1371/journal.pone.0285418.s005
DOI:
10.1371/journal.pone.0285418.s006
DOI:
10.1371/journal.pone.0285418.s007
DOI:
10.1371/journal.pone.0285418.s008
DOI:
10.1371/journal.pone.0285418.s009
DOI:
10.1371/journal.pone.0285418.s010
DOI:
10.1371/journal.pone.0285418.s011
DOI:
10.1371/journal.pone.0285418.s012
DOI:
10.1371/journal.pone.0285418.s013
DOI:
10.1371/journal.pone.0285418.r001
DOI:
10.1371/journal.pone.0285418.r002
DOI:
10.1371/journal.pone.0285418.r003
DOI:
10.1371/journal.pone.0285418.r004
DOI:
10.1371/journal.pone.0285418.r005
DOI:
10.1371/journal.pone.0285418.r006
DOI:
10.1371/journal.pone.0285418.r007
DOI:
10.1371/journal.pone.0285418.r008
Sprache:
Englisch
Verlag:
Public Library of Science (PLoS)
Publikationsdatum:
2023
ZDB Id:
2267670-3
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