Danon Disease-Associated LAMP-2 Deficiency Drives Metabolic Signature Indicative of Mitochondrial Aging and Fibrosis in Cardiac Tissue and hiPSC-Derived Cardiomyocytes

Giorgia Del Favero (Corresponding author), Alois Bonifacio, Teisha J. Rowland, Shanshan Gao, Kunhua Song, Valter Sergo, Eric D. Adler, Luisa Mestroni, Orfeo Sbaizero, Matthew R. G. Taylor (Corresponding author)

Publications: Contribution to journalArticlePeer Reviewed

Original languageEnglish
Article number2457
Pages (from-to)1-20
Number of pages20
JournalJournal of clinical medicine
Volume9
Issue number8
DOIs
Publication statusPublished - Aug 2020

Austrian Fields of Science 2012

  • 301305 Medical chemistry

Keywords

  • Danon disease
  • LAMP-2 deficiency
  • cardiac fibrosis
  • multi-omics profiling
  • mitochondrial aging phenotype
  • cell biomechanics
  • HUMAN FIBROBLASTS
  • CARDIOMYOPATHY
  • HOMEOSTASIS
  • EXPRESSION
  • AUTOPHAGY
  • FEATURES
  • PATHWAY
  • OPOSSUM
  • DAMAGE
  • TOOLS
  • Mitochondrial aging phenotype
  • Multi-omics profiling
  • Cardiac fibrosis
  • Cell biomechanics

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