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. 2024 Feb 8;19(3):366–382. doi: 10.1016/j.stemcr.2024.01.002

Figure 1.

Figure 1

Characterization of IPSC-derived motor neurons

(A) Two isogenic iPSC models were generated using gene editing to introduce the R155H mutation into the p97 endogenous locus (KOLF2.1) and correct the R155H mutation (392.1) to wild type.

(B) Phase contrast images of iPSCs (top row), embryoid bodies (middle row), and day 30 motor neurons (bottom row). Scale bars, 50 μm (top and bottom rows) and 100 μm (middle row).

(C) Schematic of the motor neuron differentiation protocol.

(D) Immunoblot of iPSCs, NPCs, and day 30 motor neurons. The faster migration band in PAX6 immunoblots likely represents another isoform.

(E) qPCR of wild-type, heterozygous, and homozygous KOLF2.1 iPSCs, NPCs, and motor neurons and markers for each stage. CHIR, CHIR99021; EB, embryoid body; LDN, LDN193189; MN, motor neuron; NPC, neural precursor cell; NTFs, neurotrophic factors; RA, retinoic acid; SA, smoothened agonist; SB, SB431542. See also Figures S1 and S2.