期刊
NEUROSCIENCE
卷 323, 期 -, 页码 195-206出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.neuroscience.2015.05.061
关键词
astrocytes; lysosomal storage diseases; mitochondrial dysfunction; neurodegeneration; reactive astrocytosis
资金
- National Institute of Neurological Disorders and Stroke (NINDS) [1R21NS084392-01A1]
Lysosomal storage diseases (LSDs) encompass a wide range of disorders characterized by inborn errors of lysosomal function. The majority of LSDs result from genetic defects in lysosomal enzymes, although some arise from mutations in lysosomal proteins that lack known enzymatic activity. Neuropathological abnormalities are a feature of several LSDs and when severe, represent an important determinant in disease outcome. Glial dysfunction, particularly in astrocytes, is also observed in numerous LSDs and has been suggested to impact neurodegeneration. This review will discuss the potential role of astrocytes in LSDs and highlight the possibility of targeting glia as a beneficial strategy to counteract the neuropathology associated with LSDs. This article is part of a Special Issue entitled: Astrocyte-Neuron Interact. (C) 2015 IBRO. Published by Elsevier Ltd. All rights reserved.
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