Dysregulation of the NLRP3 Inflammasome and Promotion of Disease by IL-1β in a Murine Model of Sandhoff Disease

Dysregulation of the NLRP3 Inflammasome and Promotion of Disease by IL-1β in a Murine Model of Sandhoff Disease

Sandhoff disease (SD) is a progressive neurodegenerative lysosomal storage disorder characterized by GM2 ganglioside accumulation as a result of mutations in the <i>HEXB</i> gene, which encodes the β-subunit of the enzyme β-hexosaminidase. Lysosomal storage of GM2 triggers inflammation i...

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Bibliographic Details
Main Authors: Nick Platt, Dawn Shepherd, David A. Smith, Claire Smith, Kerri-Lee Wallom, Raashid Luqmani, Grant C. Churchill, Antony Galione, Frances M. Platt
Format: Article
Language:English
Published: MDPI AG 2025-01-01
Series:Cells
Subjects:
Sandhoff disease
GM2 gangliosidosis
lysosomal storage disease
NLRP3 inflammasome
IL-1β
inflammation
Online Access:https://www.mdpi.com/2073-4409/14/1/35
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