Table 1.
Highlighted mouse lines contributing to study of cell-autonomous and non-cell-autonomous impact of mHtt on HD-like phenotypes
| Pan-cellular | ||||||||
| Motor deficits | Inclusions | Neuronal loss | Abnormal white matter | Microglial/astroglial activation | Transcriptional abnormalities | Mitochondrial | Corticostriatal synapse abnormalities | |
| R6 [24] | Yes | Yes | Absent [25] | Yes | Yes | Yes | Yes | Yes [26] |
| YAC [27] | Yes | Yes | Yes | Yes [28] | Yes [29] | Yes [30] | Yes [31] | Yes |
| BAC [32] | Yes | Yes | Presence of dark neurons | Yes | Not evaluated | Yes | Not evaluated | Yes [33] |
| Pan-neuronal | ||||||||
| Motor deficits | Inclusions | Neuronal loss | Abnormal white matter | Microglial/astroglial activation | Transcriptional abnormalities | Mitochondrial | Corticostriatal synapse abnormalities | |
| Prion [34] | Yes | Yes | Yes | Not evaluated | Astrogliosis | Yes | Yes | Not evaluated |
| RosaHD/Nestin-Cre [35] | Yes | Yes | Dark neurons | Not evaluated | Astrogliosis | Not evaluated | Not evaluated | Yes |
| Neuronal subtype-specific | ||||||||
| Motor deficits | Inclusions | Neuronal loss | Abnormal white matter | Microglial/astroglial activation | Transcriptional abnormalities | Mitochondrial | Corticostriatal synapse abnormalities | |
| D9-N171-82Q [36] | Yes | Yes | No striatal volume loss | Not evaluated | Not evaluated (no astrogliosis) | Yes | Yes [37] | Yes [37] |
| RosaHD/Emx1-Cre [35] | Absent | Yes | Absent (only degeneration vacuoles) | Not evaluated | Not evaluated (no astrogliosis) | Not evaluated | Not evaluated | No |
| mHtt neuronal subtype-specific deletions | ||||||||
| Motor deficits | Inclusions | Neuronal loss | Abnormal white matter | Microglial/astroglial activation | Transcriptional abnormalities | Mitochondrial | Corticostriatal synapse abnormalities | |
| BACHD/Rgs9-Cre [38] | Yes | Yes | Not evaluated | Not evaluated | Not evaluated | Not evaluated | Not evaluated | Yes? |
| BACHD/Emx1-Cre [38] | Improved | Not evaluated | Not evaluated | Not evaluated | Not evaluated | Not evaluated | Not evaluated | No |
| BACHD/Nestin-Cre [35] | Improved | Not evaluated | Recovered striatal volume | Not evaluated | Yes | Not evaluated | Not evaluated | Not evaluated |
| Non-neuronal subtype-specific deletions | ||||||||
| Motor deficits | Inclusions | Neuronal loss | Abnormal white matter | Microglial activation | Transcriptional abnormalities | Mitochondrial | Corticostriatal synapse abnormalities | |
| BACHD/GFAP-CreERT2 [39] | Improved | Not evaluated | Partial recovery of striatal volume | Not evaluated | Not evaluated | Not evaluated | Not evaluated | No |
| BACHD/Lyz2 [40] | Yes | Not evaluated | No recovery of striatal volume | Not evaluated | Absent | Not evaluated | Not evaluated | Not evaluated |
| BACHD/NG2-Cre [41] | Improved | Not evaluated | No recovery striatal volume | No | Not evaluated | Improved | Not evaluated | Not evaluated |
| Non-neuronal specific overexpressers | ||||||||
| Motor deficits | Inclusions | Neuronal loss | Abnormal white matter | Microglial activation | Transcriptional abnormalities | Mitochondrial | Corticostriatal synapse abnormalities | |
| GFAP/160Q [42] | Yes | Yes | No | Not evaluated | Not evaluated | qPCR for specific astroglial genes | Not evaluated | Not evaluated |
| RosaHD/Cx3cr1-Cre [43] | Not evaluated | Not evaluated | Not evaluated | Not evaluated | Yes | Yes | Not evaluated | Not evaluated |
| Plp-150Q [44] | Yes | Yes | Not evaluated | Yes | Not evaluated | Yes | Not evaluated | Not evaluated |