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β-Galactosylceramidase Deficiency Causes Upregulation of Long Pentraxin-3 in the Central Nervous System of Krabbe Patients and Twitcher Mice
Globoid cell leukodystrophy (GLD), or Krabbe disease, is a neurodegenerative sphingolipidosis caused by genetic deficiency of lysosomal β-galactosylceramidase (GALC), characterized by neuroinflammation and demyelination of the central (CNS) and peripheral nervous system. The acute phase protein long...
Autores principales: | Coltrini, Daniela, Chandran, Adwaid Manu Krishna, Belleri, Mirella, Poliani, Pietro L., Cominelli, Manuela, Pagani, Francesca, Capra, Miriam, Calza, Stefano, Prioni, Simona, Mauri, Laura, Prinetti, Alessandro, Kofler, Julia K., Escolar, Maria L., Presta, Marco |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9409448/ https://www.ncbi.nlm.nih.gov/pubmed/36012705 http://dx.doi.org/10.3390/ijms23169436 |
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