Lysosomal Storage Diseases-Regulating Neurodegeneration

Lysosomal Storage Diseases-Regulating Neurodegeneration

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Autophagy is a complex pathway regulated by numerous signaling events that recycles macromolecules and can be perturbed in lysosomal storage diseases (LSDs). The concept of LSDs, which are characterized by aberrant, excessive storage of cellular material in lysosomes, developed following the discove...

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Main Authors: Rob U. Onyenwoke, Jay E. Brenman
Format: Article
Language:English
Published: SAGE Publishing 2015-01-01
Series:Journal of Experimental Neuroscience
Online Access:https://doi.org/10.4137/JEN.S25475
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spelling doaj-7a1a3e05f8d746faa5e1f7c57a4af89c2020-11-25T02:47:50ZengSAGE PublishingJournal of Experimental Neuroscience1179-06952015-01-019s210.4137/JEN.S25475Lysosomal Storage Diseases-Regulating NeurodegenerationRob U. Onyenwoke0Jay E. Brenman1 Department of Pharmaceutical Science, Biomanufacturing Research Institute and Technology Enterprise (BRITE), North Carolina Central University, Durham, NC, USA. Neuroscience Center, The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.Autophagy is a complex pathway regulated by numerous signaling events that recycles macromolecules and can be perturbed in lysosomal storage diseases (LSDs). The concept of LSDs, which are characterized by aberrant, excessive storage of cellular material in lysosomes, developed following the discovery of an enzyme deficiency as the cause of Pompe disease in 1963. Great strides have since been made in better understanding the biology of LSDs. Defective lysosomal storage typically occurs in many cell types, but the nervous system, including the central nervous system and peripheral nervous system, is particularly vulnerable to LSDs, being affected in two-thirds of LSDs. This review provides a summary of some of the better characterized LSDs and the pathways affected in these disorders.https://doi.org/10.4137/JEN.S25475
collection DOAJ
language English
format Article
sources DOAJ
author Rob U. Onyenwoke
Jay E. Brenman
spellingShingle Rob U. Onyenwoke
Jay E. Brenman
Lysosomal Storage Diseases-Regulating Neurodegeneration
Journal of Experimental Neuroscience
author_facet Rob U. Onyenwoke
Jay E. Brenman
author_sort Rob U. Onyenwoke
title Lysosomal Storage Diseases-Regulating Neurodegeneration
title_short Lysosomal Storage Diseases-Regulating Neurodegeneration
title_full Lysosomal Storage Diseases-Regulating Neurodegeneration
title_fullStr Lysosomal Storage Diseases-Regulating Neurodegeneration
title_full_unstemmed Lysosomal Storage Diseases-Regulating Neurodegeneration
title_sort lysosomal storage diseases-regulating neurodegeneration
publisher SAGE Publishing
series Journal of Experimental Neuroscience
issn 1179-0695
publishDate 2015-01-01
description Autophagy is a complex pathway regulated by numerous signaling events that recycles macromolecules and can be perturbed in lysosomal storage diseases (LSDs). The concept of LSDs, which are characterized by aberrant, excessive storage of cellular material in lysosomes, developed following the discovery of an enzyme deficiency as the cause of Pompe disease in 1963. Great strides have since been made in better understanding the biology of LSDs. Defective lysosomal storage typically occurs in many cell types, but the nervous system, including the central nervous system and peripheral nervous system, is particularly vulnerable to LSDs, being affected in two-thirds of LSDs. This review provides a summary of some of the better characterized LSDs and the pathways affected in these disorders.
url https://doi.org/10.4137/JEN.S25475
work_keys_str_mv AT robuonyenwoke lysosomalstoragediseasesregulatingneurodegeneration
AT jayebrenman lysosomalstoragediseasesregulatingneurodegeneration
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