Table 1.
Modeling SBMA—in vitro models
| Cell model | Species | Cell type | PolyQ length | Aggregation | Hormone-dependent cell death | Reference |
|---|---|---|---|---|---|---|
| PC12 | Rat | Pheochromocytoma-derived | 10Q/112Q | Yes | Yes | [161] |
| NSC-34 | Mouse | Motor neuron-derived | 0Q/23Q/46Q | Yes | No | [165] |
| MN-1 | Mouse | Motor neuron-derived | 24Q/65Q/100Q | No | No | [123, 163] |
| C2C12 | Mouse | Immortalized myoblasts | 24Q/100Q | No | No | [170] |
| iPSC-derived NPC | Human | Neuronal precursor cells | Varied | Yes | No | [168] |
| Mesenchymal stem cells | Human | Adipose-derived | Varied | Yes* | No | [173] |
SBMA cell models are further reviewed by Pennuto and Basso [225]
*Mesenchymal stem cells from SBMA patients form aggregates upon proteasomal inhibition via treatment with MG132