Altered Inflammatory Signature in a C9ORF72 -ALS iPSC-Derived Motor Neuron and Microglia Coculture Model.

ABSTRACT: Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disorder involving multiple cell types in the central nervous system. The key pathological features of ALS include the degeneration of motor neurons and the initiation and propagation of neuroinflammation mediated by nonneuronal cell types such as microglia. Currently, the specific mechanisms underlying the involvement of microglia in neuroinflammation in ALS are unclear. Consequently, we generated several human-induced pluripotent stem cell (iPSC) derived motor neuron and microglia cocultures. We utilized ALS patient-derived iPSCs carrying a common genetic variant, the hexanucleotide repeat expansion (HRE) in C9ORF72, as well as C9ORF72 knockout (KO) iPSC lines. iPSC-derived motor neurons and microglia demonstrated express

SUBMITTER: Gao Y 

PROVIDER: S-EPMC12667000 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature