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Type 1 Fucosidosis Is a Rare Human Disease α\alpha -1-Fucosidase),and Is Inherited in an Autosomal Recessive Manner

Question 17

Multiple Choice

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\alpha -1-fucosidase) ,and is inherited in an autosomal recessive manner.Which of the following statements is ACCURATE about Type1 fucosidosis?


A) Ff and ff individuals will have the disease
B) Ff individuals will have the disease,because anyone with the mutant allele will have the disease
C) Ff individuals make enough α\alpha -1-fucosidase to break down fucose and do not exhibit the condition
D) Two carriers for Type I fucosidosis have no chance of having a normal child
E) Both Ff individuals will have the disease,because anyone with the mutant allele will have the disease AND two carriers for Type I fucosidosis have no chance of having a normal child

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