Purification and characterization of altered cystic fibrosis liver alpha-L-fucosidase.
Publication/Presentation Date
5-1-1978
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.
Volume
13
Issue
5
First Page
417
Last Page
424
ISSN
0009-9163
Published In/Presented At
Alhadeff, J. A., Watkins, P., & Freeze, H. (1978). Purification and characterization of altered cystic fibrosis liver alpha-L-fucosidase. Clinical genetics, 13(5), 417–424. https://doi.org/10.1111/j.1399-0004.1978.tb04141.x
Disciplines
Medicine and Health Sciences
PubMedID
657582
Department(s)
Department of Medicine
Document Type
Article