Isoenzymes of N-acetyl-beta-hexosaminidase.

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Separation of three isoenzymes of N-acetyl-beta-D-hexosaminidase from human tissues by cellulose acetate membrane electrophoresis.

The separation of N-acetyl-beta-D-hexosaminidase isoenzymes from human tissues is used in the diagnosis and differential diagnosis of GM(2) gangliosidosis, since in type 1 the A isoenzyme is deficient and in type 2 both the A and B isoenzymes are deficient. Peripheral blood leucocytes are commonly used for these investigations, and the present study demonstrates that, in addition to these two i...

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A study of highly purified mucolipidosis III urinary N-acetyl-beta-D-hexosaminidase B.

Highly purified N-acetyl-beta-D-hexosaminidase B from normal urine and urine of a patient with mucolipidosis III was used to determine whether it has undergone any of the alterations associated with this genetic defect. Examination by sodium dodecyl sulphate/polyacrylamide gel electrophoresis showed that both the enzyme preparations contained protein components with apparent Mr values of 55 000...

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Marked elevation of serum N-acetyl-beta-D-hexosaminidase activity in rheumatoid rheumatoid arthritis.

OBJECTIVE To study N-acetyl-beta-D-hexosaminidase (NAHase) activity in the sera of rheumatoid arthritis (RA) patients and to determine its source. METHODS NAHase activity in the serum and synovial fluid of RA patients was measured with p-nitrophenyl beta-N-acetylglucosaminide as substrate. The p-nitrophenol released was measured spectrophotometrically in an ELISA reader. Rabbit articular chon...

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Role of N-acetyl-β-d-hexosaminidase in cholesteatoma tissue

Cholesteatoma is a destructive disease characterized by the progressive expansion of keratinizing squamous epithelium in the middle ear and mastoid, and chronic inflammatory reaction of the subepithelial connective tissue. N-Acetyl-β-d-hexosaminidase (HEX) catalyzes the release of terminal non-reducing N-acetyl-d-hexosamine residues acting on glucosides and galactosides in glycoproteins, GM2-ga...

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Activity of N-acetyl-beta-D-hexosaminidase (HEX) and its isoenzymes A and B in human milk during the first 3 months of breastfeeding.

PURPOSE Milk contains free and bound oligo- and heteropolisaccharides, which protect newborns against pathogens and have nutritional value. N-acetyl-beta-D-hexosaminidase (HEX), the most active lysosomal exoglycosidase, modify and degrade oligo- and heteropolysaccharides. The objective of our study was to determine HEX activity and isoenzymes A and B in the progression of lactation. MATERIAL ...

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ژورنال

عنوان ژورنال: Acta Biochimica Polonica

سال: 1999

ISSN: 1734-154X,0001-527X

DOI: 10.18388/abp.1999_4146