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In this study, we inactivated <jats:italic>Celsr3<\/jats:italic> in the brainstem using En1-Cre mice (<jats:italic>Celsr3<\/jats:italic> cKO) and analyzed axonal and behavioral phenotypes. <jats:italic>Celsr3<\/jats:italic> cKO animals showed an 83% reduction of rubrospinal axons and 30% decrease of corticospinal axons in spinal segments, associated with increased branching of dopaminergic fibers in the ventral horn. Decreases of spinal motoneurons, neuromuscular junctions, and electromyographic signal amplitude of the biceps were also found in mutant animals. Mutant mice had impaired motor coordination and defective response to heavy mechanical stimulation, but no disability in walking and food pellet handling. Transsynaptic tracing demonstrated that rubrospinal axons synapse on spinal neurons in the deep layer of the dorsal horn, and mechanical stimulation of hindpaws induced strong calcium signal of red nuclei in control mice, which was less prominent in mutant mice. 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