Fig. 3.

Use of patient-derived iPSC in modeling Alexander disease. Somatic cells obtained from AxD patients are reprogrammed to their pluripotent state using specific transcription factors. Neurons and astrocytes differentiated from these iPSCs, aid in recapitulating the relevant disease phenotype showing unique cytoplasmic inclusions called Rosenthal fibers in astrocytes, reduced myelination, and increased inflammatory response. These iPS cells serve as a promising platform to evaluate the efficacy of various therapeutic drugs and personalized drug treatment.