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A Selective Inhibitor Gal‐PUGNAc of Human Lysosomal β‐Hexosaminidases Modulates Levels of the Ganglioside GM2 in Neuroblastoma Cells [PDF]
Angewandte Chemie - International Edition, 2009 Keith A Stubbs +2 more
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Hexosaminidases: Multiple Component Enzymes
1976In 1968 Robinson and Stirling (1) demonstrated that lysosomal N-acetylhexosaminidase could be separated into two species by starch gel electrophoresis. They noted that the more anodic form, A, was heat-labile and that it could be converted to the less anodic form, B, by the action of neuraminidase.
J A, Lowden, J W, Callahan, F N, Howard
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Hexosaminidase A deficiency in adults
American Journal of Medical Genetics, 1986AbstractDeficiency of hexosaminidase A (Hex A) in adults was found in 15 individuals from nine unrelated Ashkenazi families; 14 individuals had neurological symptoms, one was clinically intact. Clinical, biochemical and genetic findings are reported and compared to previously reported cases.The clinical picture varied between and within families and ...
Ruth Navon +4 more
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Differential inhibition of β-hexosaminidase A and β-hexosaminidase B by suramin
Biochemical and Biophysical Research Communications, 1981Abstract The trypanocidal drug suramin is a potent inhibitor of β-hexosaminidase A with a K i of about 4.5 μM, and to a lesser extent of β-hexosaminidase B (K i 31.5 μM). β-Hexosaminidase B remained active in the presence of 1.0 mM suramin whereas the activity of β-hexosaminidase A was completely inhibited at 0.1 mM.
G, Constantopoulos +4 more
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Antibody against purified human hexosaminidase B cross-reacting with human hexosaminidase A
Biochemical and Biophysical Research Communications, 1972Abstract Human hexosaminidase B has been purified to virtually homogeneous state from placenta. An anti-serum has been prepared in rabbits against the purified preparation. The serum reacted equally with human hexosaminidase B (free of hexosaminidase A) and with human hexosaminidase A (free of hexosaminidase B) as shown by immunodiffusion and by ...
S K, Srivastava, E, Beutler
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A structural difference between the β-chains in hexosaminidase B and hexosaminidase A
Canadian Journal of Biochemistry, 1981Hexosaminidase (Hex) BA was prepared from purified placental Hex A by treatment with merthiolate and rechromatography on DEAE Sepharose 6B-CL. The heat stability, isoelectric focussing pattern, and peptide map of this isoenzyme were compared with those of naturally occurring purified placental Hex B. Hex BA proved to be less heat stable at 60 °C, have
D, Mahuran, J A, Lowden
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Serum β-hexosaminidases in pregnancy
Clinica Chimica Acta, 1979Serum contains several hexosaminidase forms. Two, HEX A and HEX B, migrate in both DEAE cellulose and isoelectric focussing systems like their tissue counterparts. A major group of serum hexosaminidases migrate in an intermediate position. These forms, collectively named HEX 1, increase during pregnancy and obscure carrier detection for Tay-Sachs and ...
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Anchimeric assistance in hexosaminidases
Canadian Journal of Chemistry, 2002Configuration retaining glycosidases catalyse the hydrolysis of glycosidic bonds via a double displacement mechanism, typically involving two key active site carboxyl groups (Glu or Asp). One of the enzymic carboxyl groups functions as a general acidbase catalyst, the other acts as a nucleophile. Alternatively, configuration-retaining hexosaminidases
Brian L Mark, Michael NG James
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[Hexosaminidase A-hexosaminidase B complex from human kidneys].
Biokhimiia (Moscow, Russia), 1991Isolation and purification on human kidney hexosaminidases A and B (EC 3.2.1.30) were carried out. The regulation of the supramolecular organization and catalytic activity of the hexosaminidases in the AOT reversed micellar system modelling the enzyme microenvironment in the lysosomes, were investigated.
A V, Pshezhetskiĭ +3 more
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Hexosaminidase isoenzyme profiles in leukemic cells
Clinica Chimica Acta, 1983The isoenzyme profiles of hexosaminidase in leukemic cells from 39 patients were examined with DEAE-Sephadex chromatography. There was a clear difference in the isoenzyme composition between normal lymphocytes and granulocytes. In acute non-T/non-B lymphocytic leukemia (ALL) a characteristic alteration was found in the intermediate forms of ...
T, Tanaka +5 more
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