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Refdoc est un service / is powered by découvrir / discover refdoc export to mendeley to endnote inist-cnrs 2, allée du parc de brabois cs 10310f-54519 vandoeuvre-lès-nancycedex france phone: +33 (0)3 83 50 46 64 fax: +33 (0)3 83 50 46 66 nous contacter contact us faire une nouvelle recherchemake a new search commander ce document order this document titre du document / document title biochemical characterization of the gm2 gangliosidosis b1 variant auteur(s) / author(s) tutorâ j. generic viagra 50mg
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generic viagra online india ; rã©sumã© / abstractthe deficiency of the a isoenzyme of î²-hexosaminidase (hex) produced by different mutations of the gene that codes for the a subunit (tay-sachs disease) has two variants with enzymological differences: the b variant consists of the absence of hex a isoenzyme and the bl variant produces an inactive hex a isoenzyme for the hydrolysis of the gm2 ganglioside and synthetic substrates with negative charge.
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In contrast to the early childhood form of the b variant, the b 1 variant appears at a later clinical stage (3 to 7 years of age) with neurodegencrative symptoms leading to the death of the patient in the second decade of life.
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The most frequent mutation responsible for the gm2 gangliosidosis b1 variant is r178h, which has a widespread geographic and ethnic distribution.
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viagra xanax drug interactions The highest incidence has been described in portugal, which has been suggested as the point of origin of this mutation.
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viagra triangle area Biochemical characterization of this lysosomal disease is carried out using negatively charged synthetic a subunit-specific sulfated substrates, since hex a isoenzyme heat-inactivation assays are not applicable. can get viagra my doctor uk generic viagra 50mg However, the determination of the apparent activation energy of hex using the neutral substrate 3,3'-dichlorophenolsulfonphthaleinyl n-acetyl-î²-d-glucosaminide, may offer a valid alternative. where can i buy viagra in hong kong
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The presence of an a subunit in the î±î² heterodimer hex a means that its activation energy (41.
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8 kj/mol) is significantly lower than that of the î²î² homodimer hex b (75. generic viagra 50mg 1 kj/mol); however, as mutetion inactivates the a subunit, the hex a of the b1 variant presents an activation energy that is similar to that of the hex b isoenzyme.
Revue / journal title brazilian journal of medical and biological research â â issnâ 0100-879xâ â codenâ bjmrdkâ source / source2004,â vol..
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