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Hexosaminidase

Purified by Sephadex G-200 filtration and DEAE-cellulose column chromatography. Hexosaminidase A was further purified by DEAE-cellulose column chromatography, followed by an ECTEOLA-cellulose column, Sephadex-200 filtration, electrofocusing and Sephadex G-200 filtration. Hexosaminidase B was purified by a CM-cellulose column, electrofocusing and Sephadex G-200 filtration. [Srivastava et al. 7 Biol Chem 249 2034 1974.]... [Pg.506]

GM2 gangliosidosis (also known as Tay-Sachs disease) is a rare disorder caused by mutations in the gene encoding the lysosomal, heterodimeric ss-hexosaminidase... [Pg.557]

GM2-gangl iosidosis Lysosomal, heterodimeric ss-hexosaminidase A (Hex A) Pyrimethamine... [Pg.1018]

Tay-Sachs disease Hexosaminidase A Cer—Gic—Gal(NeuAc)-i-GalNAc GM2Gangiioside Mental retardation, blindness, muscular weakness. [Pg.203]

Taracha E, Habrat BS, Wozniak P, Walkowiak J and Szukalski B (2001). The activity of (3-hexosaminidase (uHex) and y-glutamyltransferase (uGGT) in urine as non invasive markers of alcohol abuse I. Alcohol-dependent subjects. World Journal of Biological Psychiatry, 2, 184-189. [Pg.285]

Sandhoff s disease GMj-ganglioside asialo-G -ganglioside, globoside Hexosaminidases A and B... [Pg.686]

Shulman, M.L., Lakhtina, O.E. and Khorlin, A.Y., Specific irreversible inhibition of human and boar 7/-acetyl-/ -D-hexosaminidase by 2-acetamido-2-deoxy-/3-D-glucopyranosyl isothiocyanate. Biochem. Biophys. Res. Commun., 1977, 74, 455. [Pg.235]

Tay-Sachs Hexosaminidase A Ganglioside GMj Cherry red spots in macula Blindness Psychomotor retardation Death usually <2 years... [Pg.234]

This enzyme [EC 3.2.1.52] (also known as /3-A-acetylhex-osaminidase, )3-hexosaminidase, hexosaminidase, and N-acetyl-/3-glucosaminidase) catalyzes the hydrolysis of terminal nonreducing A-acetyl-o-hexosamine residues in... [Pg.339]

OCTAHEDRAL COORDINATION HEXA UNI PING PONG ENZYME MECHANISM HEXOKINASE ATP GTP DEPLETION DEINHIBITION INDUCED FIT HYPOTHESIS HEXOSAMINIDASE A and B... [Pg.748]

Figure 7. Multiclass NaTve Bayes modeling within Pipeline Pilot software (www.scitegic.com) based on the WOMBAT chemogenomics dataset Probabilistic target predictions are possible for compounds given only their chemical structure. In the example shown, the WOMBAT targets were predicted for Calphostin C, a known protein kinase C inhibiting natural product Tubulin and beta-hexosaminidase are predicted as additional possible targets. Figure 7. Multiclass NaTve Bayes modeling within Pipeline Pilot software (www.scitegic.com) based on the WOMBAT chemogenomics dataset Probabilistic target predictions are possible for compounds given only their chemical structure. In the example shown, the WOMBAT targets were predicted for Calphostin C, a known protein kinase C inhibiting natural product Tubulin and beta-hexosaminidase are predicted as additional possible targets.
The crystal structure of human P-hexosaminidase B (GH 20) in its complex with these inhibitors (GlcNAc-thiazoline 140 PDB 1NP0 GalNAc-isofagomine 139 PDB 1NOW) revealed a very similar picture, with an activated water molecule above the P face ready to attack the anomeric center. 300... [Pg.236]

O-GlcNAcase with even higher selectivities of up to 9 x 105-fold over the lysosomal hexosaminidases.306,307 The A/-(3-thiopropanoyl) derivative 145 in complex with recombinant enzyme (PDB 2XPK) showed the acyl residue in a selectivity pocket, which distinguishes the enzyme from the human lysosomal hexosaminidases, which feature more shallow pockets that cannot accommodate the extended acyl moiety. [Pg.238]

B. L. Mark, D. J. Mahuran, M. M. Chemey, D. Zhao, S. Knapp, and M. N. G. James, Crystal structure of human (i-hexosaminidase B Understanding the molecular basis of Sandhoff and Tay-Sachs... [Pg.292]

H. C. Dorfmueller, V. S. Borodkin, M. Schimpl, X. Zheng, R. Kime, K. D. Read, and D. M. F. van Aalten, Cell-penetrant, nanomolar O-GlcNAcase inhibitors selective against lysosomal hexosaminidases, Chem. Biol., 17 (2010) 1250-1255. [Pg.292]

See other pages where Hexosaminidase is mentioned: [Pg.477]    [Pg.506]    [Pg.54]    [Pg.532]    [Pg.219]    [Pg.563]    [Pg.136]    [Pg.686]    [Pg.687]    [Pg.689]    [Pg.318]    [Pg.319]    [Pg.321]    [Pg.321]    [Pg.325]    [Pg.252]    [Pg.63]    [Pg.95]    [Pg.235]    [Pg.348]    [Pg.339]    [Pg.28]    [Pg.193]    [Pg.212]    [Pg.237]    [Pg.239]    [Pg.269]    [Pg.269]    [Pg.269]    [Pg.269]    [Pg.269]    [Pg.280]    [Pg.291]   
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Hexosamines Hexosaminidase

Hexosaminidase A and

Hexosaminidases

Hexosaminidases

Lysosomal enzyme hexosaminidase

P-Hexosaminidase A and

P-Hexosaminidases

P-hexosaminidase

Tay-Sachs disease, hexosaminidases

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