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. 2012 Aug 30;3(8):e382. doi: 10.1038/cddis.2012.121

Table 1. Examples of current HD in vitro models.

Cell lines Constructs Aggregate localization Observations/Results References
HEK293T Q15 and Q128 Cytoplasm and nucleus Toxicity is more affected by the rate of aggregate occurrence than their localization Hackam et al.43
SK-N-SK pFlag-GFP exon 1-Q21 or Q72 and co-transfection with full-length Htt NA Full-length Htt reduces cell death in mHtt-expressing cells, but has no effect on inclusions Ho et al.131
PC12 Full-length Htt 16Q, 48Q, 89Q NA mHtt expression leads to cell morphology changes Disruption of cellular signaling is mediated by NGF and EGF Song et al.132
N2A and HEK293T E15–E16 murine primary cortical neurons Q15 and Q47-Flag tag NA β-sheet structure of mHtt results in aggregate formation and cellular toxicity Poirier et al.50
ST14A Httex1Qn-GFP (n=25, 47 or 72Q) NA Probability of a cell remaining aggregate-free drops exponentially with time Colby et al.133
COS-7 or SH-SY5Y Q58 (lentivirus) Cytoplasm and nucleus Cytotoxic effects of soluble polyQ oligomers Takahashi et al.134
N2a pEGFP-C3-exon 1 20Q or 150Q Cytoplasm mHtt leads to inhibition of neurite outgrowth Higher cell death in mHtt-expressing cells Ye et al.135
N2a Q25 and Q46 fused to Cerulean or Emerald tag NA Kinetic of aggregate formation Olshina et al.136
SH-SY5Y mHtt-60Q or 150Q-EGFP expressed alone or coexpressed with ATP synthase α NA ATP synthase α suppresses the formation of insoluble Htt polyQ inclusions and reduces the toxicity induced by Htt polyQ Wang et al.137
COS-7 EGFP-tagged exon 1 with 25, 46, 97Q Cytoplasm Dynamic process of aggregate formation Suggestion of a four phase process in inclusion formation Ossato et al.52
HC2S2 EGFP-Htt exon 1-28Q or 74Q Cytoplasm and nucleus mHtt-expressing cells show nuclear fragmentation and neuritic degeneration that are time-dependent Absence of cell death when cells are undifferentiated Dong et al.138
Rat primary striatal neurons pGW1-Httex1-(Q17, Q25, Q47, Q72 or Q97) NA Investigate different antibodies to label mHtt and its various forms Miller et al.139