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Expression and Endocytosis of Lysosomal Aspartylglucosaminidase in Mouse Primary Neurons
Journal of Neuroscience
Aspartylglucosaminuria (AGU) is a neurodegenerative lysosomal storage disease that is caused by mutations in the gene encoding for a soluble hydrolase, aspartylglucosaminidase (AGA). In this study, we have used our recently developed mouse model for AGU and analyzed processing, intracellular localization, and endocytosis of recombinant AGA in telencephalic AGU mouse neurons in vitro. The processing steps of AGA were found to be similar to the peripheral cells, but both the accumulation of thedoi:10.1523/jneurosci.18-19-07750.1998 pmid:9742145 fatcat:ofirhjzpgbao7fmmrhv3j6zube