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 in the CNS an...

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Principais autores:	Nick Platt, Dawn Shepherd, David A. Smith, Claire Smith, Kerri-Lee Wallom, Raashid Luqmani, Grant C. Churchill, Antony Galione, Frances M. Platt
Formato:	Artigo
Idioma:	Inglês
Publicado em:	MDPI AG 2025-01-01
Colecção:	Cells
Assuntos:	Sandhoff disease GM2 gangliosidosis lysosomal storage disease NLRP3 inflammasome IL-1β inflammation
Acesso em linha:	https://www.mdpi.com/2073-4409/14/1/35
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