title: Fucosidosis in a family of American-bred English Springer Spaniels.
abstract: Fucosidosis was detected in a family of English Springer Spaniels in the United States. To our knowledge, these are the first reported cases of this disease in American-bred dogs. Affected and carrier status of dogs were determined by measuring the activity of the enzyme alpha-L-fucosidase in plasma and in leukocytes. Fucosidosis results in neurologic signs, particularly changes in behavior, in adolescent and adult dogs. Late onset of signs may result in misdiagnosis as a primary behavior problem or acquired neurologic disease. Fucosidosis is inherited in an autosomal recessive manner, and carrier dogs are clinically normal. Thus, the abnormal gene can become widespread in a population before homozygous-affected dogs are produced.
authors.affiliation: Department of Clinical Sciences, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins 80523, USA.
authors.forename: M O
authors.initials: MO
authors.lastname: Smith
authors.forename: D A
authors.initials: DA
authors.lastname: Wenger
authors.forename: S L
authors.initials: SL
authors.lastname: Hill
authors.forename: J
authors.initials: J
authors.lastname: Matthews
mesh_terms: D000818:Animals; D001947:Breeding; D004283:Dog Diseases / Q000175:diagnosis / Q000235:genetics*; D004285:Dogs; D005260:Female; D005645:Fucosidosis / Q000175:diagnosis / Q000235:genetics / Q000662:veterinary*; D008297:Male; D009460:Neurologic Examination / Q000662:veterinary