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Lysosomal storage disorders: The cellular impact of lysosomal dysfunction

The Journal of cell biology, 2012-11, Vol.199 (5), p.723-734 [Peer Reviewed Journal]

2012 Platt et al. 2012 ;ISSN: 0021-9525 ;EISSN: 1540-8140 ;DOI: 10.1083/jcb.201208152 ;PMID: 23185029 ;CODEN: JCLBA3

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  • Title:
    Lysosomal storage disorders: The cellular impact of lysosomal dysfunction
  • Author: Platt, Frances M. ; Boland, Barry ; van der Spoel, Aarnoud C.
  • Subjects: Cellular biology ; Homeostasis ; Metabolic disorders ; Mutation ; Proteins ; Reviews
  • Is Part Of: The Journal of cell biology, 2012-11, Vol.199 (5), p.723-734
  • Description: Lysosomal storage diseases (LSDs) are a family of disorders that result from inherited gene mutations that perturb lysosomal homeostasis. LSDs mainly stem from deficiencies in lysosomal enzymes, but also in some non-enzymatic lysosomal proteins, which lead to abnormal storage of macromolecular substrates. Valuable insights into lysosome functions have emerged from research into these diseases. In addition to primary lysosomal dysfunction, cellular pathways associated with other membrane-bound organelles are perturbed in these disorders. Through selective examples, we illustrate why the term “cellular storage disorders” may be a more appropriate description of these diseases and discuss therapies that can alleviate storage and restore normal cellular function.
  • Publisher: New York: Rockefeller University Press
  • Language: English
  • Identifier: ISSN: 0021-9525
    EISSN: 1540-8140
    DOI: 10.1083/jcb.201208152
    PMID: 23185029
    CODEN: JCLBA3
  • Source: Geneva Foundation Free Medical Journals at publisher websites
    PMC
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