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. 2021 Jun 22;10(7):1565. doi: 10.3390/cells10071565

Table 7.

Phenotypes observed in Huntington’s disease induced neural precursor cell-derived neurons.

Study HD Line Differentiation Huntington’s Disease Phenotype
Hou et al. (2017) [133] 41 CAG (onset not reported)
41 CAG (onset not reported)
Subpopulation TUJ1+, GFAP+, GALC+
  • Increased γH2AX indicating DNA damage in embryonic-like progenitors

  • Adenosine receptor agonist reversed phenotype

Monk et al. (2021) [134] 41 CAG (onset 56 years)
43 CAG (onset 50 years)
44 CAG (onset 33 years)
57 CAG (onset 27 years)
20% TUJ1+ (normal)
12% TUJ1+ (HD)
69% DARPP32+/TUJ1+
(normal)
54% DARPP32+/TUJ1+ (HD)
  • Ubiquitinated mHTT aggregates (57 CAG)

  • Maintenance of transcriptional phenotype

  • Significantly smaller cell soma areas

  • Significantly shorter neurite length

  • Significantly reduced neurite branching

  • Significantly more depolarized RMPs

  • Decreased BDNF protein correlated with reduced age of symptom onset and increased number of CAG repeats