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Deficiency of the frontotemporal dementia gene GRN results in gangliosidosis

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
Source :
Nature Communications, Vol 13, Iss 1, Pp 1-13 (2022)
Publication Year :
2022
Publisher :
Nature Portfolio, 2022.

Abstract

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.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
13
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.1474593e97d94a4ab73bc2bde059f30e
Document Type :
article
Full Text :
https://doi.org/10.1038/s41467-022-33500-9