Aberrant axon branching via Fos-B dysregulation in FUS-ALS motor neurons.

ABSTRACT:

Background

The characteristic structure of motor neurons (MNs), particularly of the long axons, becomes damaged in the early stages of amyotrophic lateral sclerosis (ALS). However, the molecular pathophysiology of axonal degeneration remains to be fully elucidated.

Method

Two sets of isogenic human-induced pluripotent stem cell (hiPSCs)-derived MNs possessing the single amino acid difference (p.H517D) in the fused in sarcoma (FUS) were constructed. By combining MN reporter lentivirus, MN specific phenotype was analyzed. Moreover, RNA profiling of isolated axons were conducted by applying the microfluidic devices that enable axon bundles to be produced for omics analysis. The relationship between the target gene, which was identified as a pathological candidate in ALS with RN

SUBMITTER: Akiyama T 

PROVIDER: S-EPMC6642224 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature