Purification and characterization of altered cystic fibrosis liver α-L-fucosidase

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Abstract
.alpha.-L-Fucosidase (EC 3.2.1.51) from 2 human cystic fibrosis livers was purified and characterized. Purification was accomplished by an affinity chromatographic procedure previously used for normal liver .alpha.-L-fucosidase. The 2 cystic fibrosis .alpha.-L-fucosidases were very similar to normal liver .alpha.-L-fucosidase with regard to pH optima profiles, Km, subunit structure and antigenicity. However, GLC analysis revealed altered carbohydrate compositions for both the cystic fibrosis .alpha.-L-fucosidases. The 3 major sugars found in normal purified liver .alpha.-L-fucosidase (mannose, N-acetylglucosamine and sialic acid) were reduced in the cystic fibrosis .alpha.-L-fucosidases, on average, to 51%, 44% and 32%, respectively, of their normal amounts.