Genetic Rodent Models of Amyotrophic Lateral Sclerosis

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.