Table 1.
Overview of spontaneous or induced mouse models showing motor neuron degeneration.
Name | Mutated gene | Gene product | Inheritance | Human disease | Reference |
---|---|---|---|---|---|
Wobbler | VPS54 | Subunit of the GARP complex | recessive | NA | [19] |
Nmd | IGHMBP2 | Immunoglobulin μ-binding protein 2 | recessive | SMARD1 | [20] |
Pmn | TBCE | tubulin-specific chaperone E | recessive | motor neuropathy HRD/SSS | [21] |
Loa | DYNC1H1 | dynactin | dominant | sensory neuropathy | [22] |
Cra | DYNC1H1 | dynactin | dominant | sensory neuropathy | [23] |
SMARD: spinal muscular atrophy with respiratory distress, HRD: hypoparathyroidism-retardation dysmorphism syndrome, SSS: Sanjad-Sakati syndrome, and NA: not available.