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Purification and characterization of altered cystic fibrosis liver α‐L‐fucosidase
Author(s) -
Alhadeff Jack A.,
Watkins Patricia,
Freeze Hud
Publication year - 1978
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1978.tb04141.x
Subject(s) - fucosidase , cystic fibrosis , sialic acid , mannose , biochemistry , protein subunit , chemistry , antigenicity , galactosamine , biology , glycoprotein , galactose , microbiology and biotechnology , immunology , fucose , antigen , gene , genetics
α‐L‐Fucosidase (E.C.3.2.1.51) from two cystic fibrosis livers has been purified and characterized. Purification was accomplished by an affinity chromatographic procedure previously used for normal liver α‐L‐fucosidase. Characterization of the two cystic fibrosis α‐L‐fucosidases indicated that they were very similar to normal liver α‐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 α‐L‐fucosidases. The three major sugars found in normal purified liver α‐L‐fucosidase (mannose, N‐acetylglucosamine and sialic acid) were reduced in the cystic fibrosis α‐L‐fucosidases, on average, to 51 %, 44 % and 32 %, respectively, of their normal amounts,