Results 171 to 180 of about 7,225 (221)

A structural difference between the β-chains in hexosaminidase B and hexosaminidase A

Canadian Journal of Biochemistry, 1981
Hexosaminidase (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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Human urinary N-acetyl-β-hexosaminidases

Biochimica et Biophysica Acta (BBA) - Enzymology, 1973
Analysis of urinary N-acetyl-β-hexosaminidases (β-2-acetamido-2-deoxy-d-glucoside acetamidodeoxyglucohydrolase, EC 3.2.1.30) by DEAE-cellulose chromatography revealed the presence of both the A and B isozymes and a new minor component designated N-acetyl-β-hexosaminidase M.
E. Grebner, J. Tucker
semanticscholar   +3 more sources

[Hexosaminidase A-hexosaminidase B complex from human kidneys].

Biokhimiia (Moscow, Russia), 1991
Isolation 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, O A, Buneeva, V M, Dvorkin, S E, Lur'e   +3 more
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Hexosaminidase isoenzyme profiles in leukemic cells

Clinica Chimica Acta, 1983
The 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, M, Kobayashi, O, Saito, N, Kamada, A, Kuramoto, T, Usui   +5 more
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Characterization of a variant of beta-hexosaminidase: "hexosaminidase Paris".

American journal of human genetics, 1977
A family (father and daughter) was found with a deficiency of hexosaminidase (HEX A and HEX B). Residual HEX A activity was about 30% of usual heterozygotes with very little HEX B activity. Thermostability of HEX A was decreased. No immunological cross reacting material was found for HEX A or B.
J C, Dreyfus, L, Poenaru, M, Vibert, N, Ravise, J, Boue   +4 more
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Crystallization of human β-hexosaminidase B

Journal of Molecular Biology, 1992
beta-Hexosaminidase is a lysosomal hydrolase that is important in the metabolism of sphingoglycolipids. beta-Hexosaminidase B and beta-hexosaminidase A are the major isozymes in normal human tissue. beta-Hexosaminidase B is a homodimer of beta subunits, and beta-hexosaminidase A is a heterodimer composed of an alpha and a beta subunit. Crystals of beta-
W B, Church, L, Swenson, M N, James, D, Mahuran   +3 more
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Structural comparison of hexosaminidases in primates

Comparative Biochemistry and Physiology Part B: Comparative Biochemistry, 1979
1. beta-Hexosaminidase (hex) structure was compared in various primates, using thin-layer isoelectric focusing on polyacrylamide gels and quantitative microcomplement fixation. 2. Isoelectric focusing revealed no intraspecies differences and similar interspecies patterns. 3.
J E, Lee, A, Yoshida, I K, Brandt
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Late‐onset Hexosaminidase A and Hexosaminidase A and B Deficiency: Family Study and Review

Developmental Medicine & Child Neurology, 1986
SUMMARYFive children from two non‐consanguineous Asian families with juvenile‐onset hexosaminidase deficiency are presented. Two have juvenile Tay‐Sachs disease with hexosaminidase A deficiency and three have juvenile Sandhoff disease with hexosaminidase A and B deficiency.
C, Adams, S, Green
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The Clinical Aspects of Adult Hexosaminidase Deficiencies

Developmental Neuroscience, 1991
The authors describe the clinical phenotypes of hexosaminidase deficiencies (GM2 gangliosidosis). The symptoms, differently combined, include cerebellar ataxia, motor neuron disease, dystonia, psychosis, neurovegetative troubles with different severity. Morphological changes are evident in rectal, muscle or nerve biopsies.
Federico, A., Palmeri, S., Malandrini, A., Fabrizi, G., Mondelli, M., Guazzi, G. C.   +5 more
openaire   +4 more sources

Revisiting glycoside hydrolase family 20 β-N-acetyl-d-hexosaminidases: Crystal structures, physiological substrates and specific inhibitors.

Biotechnology Advances, 2018
Tian Liu, Yanwei Duan, Q. Yang
semanticscholar   +1 more source