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Extracellular matrix and vascular dynamics in the kidney of a murine model for Marfan syndrome.
de Souza, Rodrigo Barbosa; Lemes, Renan Barbosa; Foresto-Neto, Orestes; Cassiano, Luara Lucena; Reinhardt, Dieter P; Meek, Keith M; Koh, Ivan Hong Jun; Lewis, Philip N; Pereira, Lygia V.
Afiliação
  • de Souza RB; Department of Genetics and Evolutionary Biology, University of São Paulo, São Paulo, SP, Brazil.
  • Lemes RB; Department of Genetics and Evolutionary Biology, University of São Paulo, São Paulo, SP, Brazil.
  • Foresto-Neto O; Faculty of Medicine, Department of Clinical Medicine, Renal Division, University of São Paulo, São Paulo, Brazil.
  • Cassiano LL; Department of Animal Sanity, Biologic Institute of São Paulo, São Paulo, SP, Brazil.
  • Reinhardt DP; Department of Anatomy and Cell Biology Dentistry and Faculty of Dental Medicine and Oral Health Sciences, McGill University, Montreal, Quebec, Canada.
  • Meek KM; Structural Biophysics Research Group, School of Optometry and Vision Sciences, Cardiff University, Cardiff, United Kingdom.
  • Koh IHJ; Department of Surgery, Federal University of São Paulo, São Paulo, SP, Brazil.
  • Lewis PN; Structural Biophysics Research Group, School of Optometry and Vision Sciences, Cardiff University, Cardiff, United Kingdom.
  • Pereira LV; Department of Genetics and Evolutionary Biology, University of São Paulo, São Paulo, SP, Brazil.
PLoS One ; 18(5): e0285418, 2023.
Article em En | MEDLINE | ID: mdl-37159453
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 mgΔlpn-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.
Assuntos

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Síndrome de Marfan Limite: Animals Idioma: En Revista: PLoS One Assunto da revista: CIENCIA / MEDICINA Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Brasil

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Síndrome de Marfan Limite: Animals Idioma: En Revista: PLoS One Assunto da revista: CIENCIA / MEDICINA Ano de publicação: 2023 Tipo de documento: Article País de afiliação: Brasil