Multiple molecular forms of certain lysosomal enzymes separated by isoelectric focusing on cellulose acetate membranes.
Analytical isoelectric focusing on cellulose acetate membranes offers greater resolution of the enzyme pattern than does standard electrophoresis and offers numerous advantages over other support media in isoelectric focusing. A series of lysosomal enzymes, deficiencies of which cause lethal storage diseases in infants and children, can be shown to exist as multiple molecular forms by analytical isoelectrical focusing on cellulose acetate membranes. This analytical technique should be a valuable tool in the investigation of enzyme polymorphism and genetically determined enzyme deficiency diseases.
D000085:Acetates; D001192:Arylsulfatases / Q000378:metabolism; D002482:Cellulose; D006596:Hexosaminidases / Q000378:metabolism; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007525:Isoelectric Focusing; D008099:Liver / Q000648:ultrastructure; D008247:Lysosomes / Q000201:enzymology*; D008567:Membranes, Artificial; D000519:alpha-Galactosidase / Q000378:metabolism
D000085:Acetates; D008567:Membranes, Artificial; D002482:Cellulose; D001192:Arylsulfatases; D006596:Hexosaminidases; D000519:alpha-Galactosidase