paperKB
coga / coga-kb
Help
Sign in

Chunk #29 — Use of iPSCs to model neurodegenerative diseases — iPSCs and amyotrophic lateral sclerosis

Source
Modeling Human Neurological and Neurodegenerative Diseases: From Induced Pluripotent Stem Cells to Neuronal Differentiation and Its Applications in Neurotrauma.
Embedded
yes

Text

Recently in 2014, researchers were able to obtain iPSCs from patients with familial forms of SOD1-mediated ALS by using lentiviral reprogramming system (Chestkov et al., 2014). Using a similar method to Dimos et al. (2008) and Chestkov et al. (2014) generated patient specific iPSC cells carrying the SOD1 mutation from primary skin fibroblasts. The resulting iPSCs expressed the same SOD1 gene mutations as the respective patients and no differences were detected among the iPSCs of the different patients with different genotypes. After 12 days, these cells were directly differentiated into motor neurons by adding RA and SHH to the culture medium. Additionally, BDNF and glial cell line-derived neurotrophic factor (GDNF) were used for the maturation of the motor neurons. These cells also expressed TUJ1 neuronal marker (Chestkov et al., 2014) but their advantage over the model of Dimos et al. (2008) was mainly due to the presence of the mTeSR1 in medium that helped in maintaining the pluripotent state needed for the unlimited production of motor neurons (Chestkov et al., 2014). That being said, it had been shown that mTeSR1