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Sex- and region-biased depletion of microglia/macrophages attenuates CLN1 disease in mice

28 Oct 2020

Abstract excerpt

Abstract Background The neuronal ceroid lipofuscinoses (CLN diseases) are fatal lysosomal storage diseases causing neurodegeneration in the CNS. We have previously shown that neuroinflammation comprising innate and adaptive immune reactions drives axonal damage and neuron loss in the CNS of palmitoyl protein thioesterase 1-deficient (Ppt1−/−) mice, a model of the infantile form of the diseases (CLN1). Therefore,...

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Glycogen Storage Diseases and Myoclonus
Lysosomal Storage Disorders Research
Neuroinflammation and Neurodegeneration Mechanisms

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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PMID: 33115477
DOI: 10.1186/s12974-020-01996-x

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