Ontology highlight
ABSTRACT:
SUBMITTER: Cougnoux A
PROVIDER: S-EPMC5985727 | biostudies-literature | 2018 Jun
REPOSITORIES: biostudies-literature
Cougnoux Antony A Drummond Rebecca A RA Collar Amanda L AL Iben James R JR Salman Alexander A Westgarth Harrison H Wassif Christopher A CA Cawley Niamh X NX Farhat Nicole Y NY Ozato Keiko K Lionakis Michail S MS Porter Forbes D FD
Human molecular genetics 20180601 12
Niemann-Pick disease, type C1 (NPC1) is a neurodegenerative disorder with limited treatment options. NPC1 is associated with neuroinflammation; however, attempts to therapeutically target neuroinflammation in NPC1 have had mixed success. We show here that NPC1 neuroinflammation is characterized by an atypical microglia activation phenotype. Specifically, Npc1-/- microglia demonstrated altered morphology, reduced levels of lineage markers and a shift toward glycolytic metabolism. Treatment with 2 ...[more]