A Gene Network Regulating Lysosomal Biogenesis and Function

DOI Web Site 被引用文献80件
  • Marco Sardiello
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Michela Palmieri
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Alberto di Ronza
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Diego Luis Medina
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Marta Valenza
    Department of Pharmacological Sciences and Center for Stem Cell Research, University of Milan, Via Balzaretti 9, 20133 Milan, Italy.
  • Vincenzo Alessandro Gennarino
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Chiara Di Malta
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Francesca Donaudy
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Valerio Embrione
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Roman S. Polishchuk
    Telethon Electron Microscopy Core Facility, Department of Cell Biology and Oncology, Consorzio Mario Negri Sud, I-66030 Santa Maria Imbaro, Chieti, Italy.
  • Sandro Banfi
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Giancarlo Parenti
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.
  • Elena Cattaneo
    Department of Pharmacological Sciences and Center for Stem Cell Research, University of Milan, Via Balzaretti 9, 20133 Milan, Italy.
  • Andrea Ballabio
    Telethon Institute of Genetics and Medicine, Via P. Castellino 111, 80131 Naples, Italy.

説明

<jats:title>Master Controller</jats:title> <jats:p> Cellular organelles allow the localized regulation of specialized processes. Under certain conditions, such as increased growth, organelles may be required to alter their function. Coordinated regulation of the gene networks required for mitochondrial and endoplasmic reticulum function has been observed. Now, <jats:bold> Sardiello <jats:italic>et al.</jats:italic> </jats:bold> (p. <jats:related-article xmlns:xlink="http://www.w3.org/1999/xlink" ext-link-type="doi" page="473" related-article-type="in-this-issue" vol="325" xlink:href="10.1126/science.1174447">473</jats:related-article> ; published online 25 June) have discovered a gene network regulating the lysosome, the major organelle involved in the degradation of internalized macromolecules. Many lysosomal genes were regulated by a single transcription factor, TFEB. TFEB itself can be activated when the lysosome malfunctions and can regulate both the abundance of lysosomes found in the cell, as well as the ability to degrade complex molecules, including a mutant protein that accumulates in patients with Huntington's disease. These results may have implications for the treatment of human lysosomal storage disorders, which are characterized by the aberrant accumulation of macromolecules causing cellular dysfunction. </jats:p>

収録刊行物

  • Science

    Science 325 (5939), 473-477, 2009-07-24

    American Association for the Advancement of Science (AAAS)

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