Altered isoenzyme patterns of liver alpha-L-fucosidase in cystic fibrosis.
Publication/Presentation Date
8-1-1976
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.
Volume
10
Issue
2NA-NA-760903-760909
First Page
63
Last Page
72
ISSN
0009-9163
Published In/Presented At
Alhadeff, J. A., Tennant, L., & O'brien, J. S. (1976). Altered isoenzyme patterns of liver alpha-L-fucosidase in cystic fibrosis. Clinical genetics, 10(2NA-NA-760903-760909), 63–72. https://doi.org/10.1111/j.1399-0004.1976.tb00014.x
Disciplines
Medicine and Health Sciences
PubMedID
954227
Department(s)
Department of Medicine
Document Type
Article