Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
6271105 | Neuroscience | 2016 | 12 Pages |
Abstract
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.
Keywords
GBATPPINCLGLDNPCPPT1S1PTripeptidyl peptidase IJNCLMSDLINCLLSDGFAPLMPDAMPGloboid cell leukodystrophyneuronal ceroid lipofuscinosisAstrocytesReactive astrocytosisMitochondrial dysfunctionSphingosine-1-phosphateDanger-associated molecular patternAlzheimer’s diseaseLysosomal storage diseaseLysosomal storage diseasesParkinson’s diseaseNeurodegenerationINCLJuvenile neuronal ceroid lipofuscinosisLate infantile neuronal ceroid lipofuscinosisInfantile neuronal ceroid lipofuscinosisNiemann-Pick type CGlial fibrillary acidic proteinPalmitoyl protein thioesteraseMultiple sulfatase deficiencyglucocerebrosidase
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Authors
K.V. Rama Rao, T. Kielian,