title: Purification and characterization of altered cystic fibrosis liver alpha-L-fucosidase.
abstract: alpha-L-Fucosidase (E.C.3.2.4.51) from two cystic fibrosis livers has been purified and characterized. Purification was accomplished by an affinity chromatographic procedure previously used for normal liver alpha-L-fucosidase. Characterization of the two cystic fibrosis alpha-L-fucosidases indicated that they were very similar to normal liver alpha-L-fucosidase with regard to pH optima profiles, Michaelis constants (Km's), subunit structure and antigenicity. However, gas liquid chromatographic analysis revealed altered carbohydrate compositions for both the cystic fibrosis alpha-L-fucosidases. The three 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.
mesh_terms: D002241:Carbohydrates / Q000032:analysis; D003550:Cystic Fibrosis / Q000201:enzymology*; D004591:Electrophoresis, Polyacrylamide Gel; D006801:Humans; D007525:Isoelectric Focusing; D008099:Liver / Q000201:enzymology*; D005644:alpha-L-Fucosidase / Q000032:analysis / Q000302:isolation & purification* / Q000378:metabolism
chemical_list: D002241:Carbohydrates; D005644:alpha-L-Fucosidase