Type 1 fucosidosis is a rare human disease. Patients cannot…
Type 1 fucosidosis is a rare human disease. Patients cannot hydrolyze the disaccharide fucose, and as a result have severe neurological decline and die by age 6. The disease is due to a defect in the gene that encodes the lysosomal enzyme that breaks down fucose (α-1-fucosidase), and is inherited in an autosomal recessive manner. Jane and John, who are both unaffected, have a child who has Type 1 fucosidosis. What is the probability that their next child will also have the disease?