Ontology highlight
ABSTRACT:
SUBMITTER: Endoh M
PROVIDER: S-EPMC8459211 | biostudies-literature | 2020 Feb
REPOSITORIES: biostudies-literature
Endoh Mitsuhiro M Baba Masaya M Endoh Tamie T Hirayama Akiyoshi A Nakamura-Ishizu Ayako A Umemoto Terumasa T Hashimoto Michihiro M Nagashima Kunio K Soga Tomoyoshi T Lang Martin M Schmidt Laura S LS Linehan W Marston WM Suda Toshio T
Cell reports 20200201 6
The tumor suppressor folliculin (FLCN) suppresses nuclear translocation of TFE3, a master transcription factor for lysosomal biogenesis, via regulation of amino-acid-sensing Rag GTPases. However, the importance of this lysosomal regulation in mammalian physiology remains unclear. Following hematopoietic-lineage-specific Flcn deletion in mice, we found expansion of vacuolated phagocytes that accumulate glycogen in their cytoplasm, phenotypes reminiscent of lysosomal storage disorder (LSD). We rep ...[more]