Deficiency of the frontotemporal dementia gene GRN results in gangliosidosis
Progranulin-deficieny results in gangliosidosis due to reduced lysosomal lipids (BMP) required for ganglioside degradation. Lysosomal ganglioside accumulation may contribute to neuroinflammation and neurodegeneration susceptibility observed in FTD.
Main Authors: | Sebastian Boland, Sharan Swarup, Yohannes A. Ambaw, Pedro C. Malia, Ruth C. Richards, Alexander W. Fischer, Shubham Singh, Geetika Aggarwal, Salvatore Spina, Alissa L. Nana, Lea T. Grinberg, William W. Seeley, Michal A. Surma, Christian Klose, Joao A. Paulo, Andrew D. Nguyen, J. Wade Harper, Tobias C. Walther, Robert V. Farese |
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Format: | Article |
Language: | English |
Published: |
Nature Portfolio
2022-10-01
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Series: | Nature Communications |
Online Access: | https://doi.org/10.1038/s41467-022-33500-9 |
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