A Gene Network Regulating Lysosomal Biogenesis and Function

• DOI: 10.1126/science.1174447
• Publication Date: 2009-06-26T01:40:27Z
• Authors: Marco Sardiello; Michela Palmieri; Alberto di Ronza; Diego Luis Medina; Marta Valenza; Vincenzo Alessand...; Chiara Di Malta; Francesca Donaudy; Valerio Embrione; Roman S. Polishchuk; Sandro Banfi; Giancarlo Parenti; Elena Cattaneo; Andrea Ballabio
• Keywords: Organelle biogenesis

Master Controller Cellular organelles allow the localized regulation of specialized processes. Under certain conditions, such as increased growth, may be required to alter their function. Coordinated gene networks for mitochondrial and endoplasmic reticulum function has been observed. Now, Sardiello et al. (p. 473 ; published online 25 June) have discovered a network regulating lysosome, major organelle involved in degradation internalized macromolecules. Many lysosomal genes were regulated by single transcription factor, TFEB. TFEB itself can activated when lysosome malfunctions regulate both abundance lysosomes found cell, well ability degrade complex molecules, including mutant protein that accumulates patients with Huntington's disease. These results implications treatment human storage disorders, which are characterized aberrant accumulation macromolecules causing cellular dysfunction.