Ontology highlight
ABSTRACT:
SUBMITTER: Yanagisawa H
PROVIDER: S-EPMC5698481 | biostudies-literature | 2017 Nov
REPOSITORIES: biostudies-literature
Yanagisawa Hiroko H Ishii Tomohiro T Endo Kentaro K Kawakami Emiko E Nagao Kazuaki K Miyashita Toshiyuki T Akiyama Keiko K Watabe Kazuhiko K Komatsu Masaaki M Yamamoto Daisuke D Eto Yoshikatsu Y
Scientific reports 20171121 1
Lysosomal storage disorders are characterized by progressive accumulation of undigested macromolecules within the cell due to lysosomal dysfunction. 573C10 is a Schwann cell line derived from a mouse model of Niemann-Pick type C disease-1, NPC (-/-). Under serum-starved conditions, NPC (-/-) cells manifested impaired autophagy accompanied by an increase in the amount of p62 and lysosome enlargement. Addition of L-leucine to serum-starved NPC (-/-) cells ameliorated the enlargement of lysosomes a ...[more]