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Editor’s note: Fucosidosis is an inherited, progressive and degenerative lysosomal disorder in humans and animals caused by the deficiency of α-l-fucosidosis. The disorder is characterized by intracellular accumulation of uncleaved storage products and results in loss of motor skills, ataxia, and mental retardation, and is lethal in early childhood. In dogs with this condition, neurological clinical signs are evident soon after birth as subtle changes in behavior, with progressive ataxia, and death at approximately 30 months of age.