Alterations in EGF-Endocytosis, Lysosomal Enzyme Transport and Maturation of Cathepsins in Juvenile Neuronal Ceroid Lipofuscinosis Fibroblasts
Background: Juvenile neuronal ceroid lipofuscinosis (JNCL), one of the most frequent forms of the NCL storage diseases, is known to be caused by loss-of-function mutations in ceroid-lipofuscinosis neuronal protein 3 (CLN3), but its cell function has not been fully elucidated. We previously reported...
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Format: | Article |
Language: | English |
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Shahid Beheshti University of Medical Sciences
2020-01-01
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Series: | International Clinical Neuroscience Journal |
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Online Access: | http://journals.sbmu.ac.ir/Neuroscience/article/download/27158/2 |