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  • Title: Altered isoenzyme patterns of liver alpha-L-fucosidase in cystic fibrosis.
    Author: Alhadeff JA, Tennant L, O'brien JS.
    Journal: Clin Genet; 1976 Aug; 10(2NA-NA-760903-760909):63-72. PubMed ID: 954227.
    Abstract:
    The isoenzyme pattern of alpha-L-fucosidase was studied by isoelectric focusing in livers from seven patients with cystic fibrosis and in normal and pathological (GM1-gangliosidosis, Type II and Sanfilippo disease) controls. The controls had very reproducible patterns consisting of seven isoenzymes of alpha-L-fucosidase with the most neutral from (I) representing a small proportion of the total activity. All seven of the cystic fibrosis livers had altered alpha-L-fucosidase isoenzyme patterns. The chemical relationship of the seven isoenzymes of normal liver alpha-L-fucosidase was investigated using neuramindase. The five most acidic forms of alpha-L-fucosidase appear to be related to the most neutral form by sialic acid residues. Since the isoenzymes of liver alpha-L-fucosidase appear to be related by sialic acid residues, it is possible that the altered alpha-L-fucosidase isoenzyme patterns found in cystic fibrosis livers may result from aberrant sialylation.
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