Figure 1

Cryoprotected MSA patient outgrowths. Scale bars are 1 cm for (A-B) and 100 μm for (C-L, O-R). (A) Gross examination of a right coronal section through the fresh brain reveals discoloration and atrophy of the globus pallidus and putamen. (B) Examination of the midbrain reveals pallor of the substantia nigra. (C) Luxol-fast blue/hematoxylin and eosin stained sections demonstrate glial cytoplasmic inclusions (Papp-Lantos bodies) in the subcortical white matter. (D) These inclusions are highlighted by immunohistochemical staining to α-synuclein. (E,I) Outgrowths with fibroblast morphology from the scalp (ASC2S-MSA; E) and dura mater (ASC2D-MSA; I). (F-H, J-L) Immunostaining of scalp (ASC2S-MSA-CP) and dura (ASC2D-MSA-CP) iPSCs demonstrates expression of pluripotency markers as indicated. AP stands for alkaline-phosphatase. DNA is in blue. (M-N) Nanostring analysis for endogenous stem cell genes (M) and shutoff of Sendai transgenes (N). Hues16 and Hues42 were used as positive controls for endogenous stem cell genes, unrelated fibroblasts as a negative control, and infected unrelated fibroblasts as a positive control for Sendai transgene expression. (O-R) Undirected EBs were cryosectioned and immunostained for the 3 developmental germ layers: endoderm (AFP), mesoderm (SMA), and ectoderm (Tuj1).