Astrocytes and lysosomal storage diseases

• Published in: Neuroscience Vol. 323; pp. 195 - 206
• Main Authors: Rama Rao, K.V, Kielian, T
• Format: Journal Article
• Language: English
• Published: United States Elsevier Ltd 26.05.2016
• Subjects: Animals; astrocytes; Astrocytes - physiology; Humans; lysosomal storage diseases; Lysosomal Storage Diseases - genetics; Lysosomal Storage Diseases - physiopathology; mitochondrial dysfunction; neurodegeneration; Neurodegenerative Diseases - genetics; Neurodegenerative Diseases - physiopathology; Neurology; Neurons - physiology; reactive astrocytosis; NPC; lysosomal storage diseases; lysosomal storage disease; juvenile neuronal ceroid lipofuscinosis; GBA; neurodegeneration; mitochondrial dysfunction; LINCL; palmitoyl protein thioesterase; JNCL; late infantile neuronal ceroid lipofuscinosis; TPPI; S1P; Niemann-Pick type C; lysosomal membrane permeability; LSD; SCMAS; MSD; AD; Alzheimer’s disease; multiple sulfatase deficiency; INCL; neuronal ceroid lipofuscinosis; glucocerebrosidase; LMP; danger-associated molecular pattern; GFAP; PD; subunit C mitochondrial ATP synthase; tripeptidyl peptidase I; infantile neuronal ceroid lipofuscinosis; NCL; PPT1; globoid cell leukodystrophy; sphingosine-1-phosphate; astrocytes; glial fibrillary acidic protein; GLD; Parkinson’s disease; reactive astrocytosis; DAMP
• ISSN: 0306-4522; 1873-7544
• DOI: 10.1016/j.neuroscience.2015.05.061

Highlights • Several lysosomal storage diseases (LSDs) are associated with CNS neuron loss. • Astrocyte dysfunction is observed in numerous LSDs and is suggested to impact neurodegeneration. • Enzyme/gene replacement in astrocytes reduces neuron loss, implicating astrocyte dysfunction in LSD pathology.