MGI:98241Mice homozygous for a null allele develop early-onset sensory and motor neuropathy with behavioral deficits, reduced compound muscle action potentials, severe distal neuromuscular junction (NMJ) denervation of fast-fatigable motor units, and intraepidermal nerve fiber loss. Heterozygous null mice show a progressive, motor-selective neuropathy with distal NMJ denervation but no sensory defects.