Journal article
Common and Uncommon Pathogenic Cascades in Lysosomal Storage Diseases
Journal of Biological Chemistry, Vol.285(27), pp.20423-20427
Jul/2010
Abstract
Lysosomal storage diseases (LSDs), of which about 50 are known, are caused by the defective activity of lysosomal proteins, resulting in accumulation of unmetabolized substrates. As a result, a variety of pathogenic cascades are activated such as altered calcium homeostasis, oxidative stress, inflammation, altered lipid trafficking, autophagy, endoplasmic reticulum stress, and autoimmune responses. Some of these pathways are common to many LSDs, whereas others are only altered in a subset of LSDs. Wenow review how these cascades impact upon LSD pathology and suggest how intervention
Details
- Title
- Common and Uncommon Pathogenic Cascades in Lysosomal Storage Diseases
- Creators
- Einat B. Vitner (null)Frances M. Platt (null)Anthony H. Futerman (null) - 972WIS_INST___112
- Resource Type
- Journal article; Review
- Publication Details
- Journal of Biological Chemistry, Vol.285(27), pp.20423-20427; Jul/2010
- Number of pages
- 5
- Language
- English
- DOI
- https://doi.org/10.1074/jbc.R110.134452
- Grant note
- estate of Louis Uger, Canada; Children's Medical Research Charity UK (Sparks); National Niemann-Pick Disease Foundation (NNPDF); System of Accelerated Research for Niemann-Pick Disease Type C (SOAR-NPC), Newlife; Action Medical ResearchThis work was supported by grants from the Children's Gaucher Research Fund, by the estate of Louis Uger, Canada (to A.H.F.), and by the Children's Medical Research Charity UK (Sparks), National Niemann-Pick Disease Foundation (NNPDF), System of Accelerated Research for Niemann-Pick Disease Type C (SOAR-NPC), Newlife, and Action Medical Research (to F.M.P.). This minireview will be reprinted in the 2010 Minireview Compendium, which will be available in January, 2011._ALMAME_DELIMITER_
- Record Identifier
- 993266086803596
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