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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5334695 | PMC |
| http://dx.doi.org/10.3390/ijerph14020141 | DOI Listing |
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Department of Population Health Sciences, University of Leicester, Leicester, UK.
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Department of Pharmacology, University of Oxford, Mansfield Road, Oxford OX1 3QT, UK.
Sandhoff disease (SD) is a progressive neurodegenerative lysosomal storage disorder characterized by GM2 ganglioside accumulation as a result of mutations in the gene, which encodes the β-subunit of the enzyme β-hexosaminidase. Lysosomal storage of GM2 triggers inflammation in the CNS and periphery. The NLRP3 inflammasome is an important coordinator of pro-inflammatory responses, and we have investigated its regulation in murine SD.
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Molecular Sciences Research Hub, Imperial College London, 82 Wood Lane, Shepherds Bush, London W12 0BZ, U.K.
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Epigenetic therapies facilitate transcription of immunogenic repetitive elements that cull cancer cells through 'viral mimicry' responses. Paradoxically, cancer-initiating events also facilitate transcription of repetitive elements. Contributions of repetitive element transcription towards cancer initiation, and the mechanisms by which cancer cells evade lethal viral mimicry responses during tumor initiation remain poorly understood.
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