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4- methylumbelliferyl- 6- sulfo- 2- acetamido- 2- deoxy- beta- glucopyranoside
Subscribe to New Research on 4-methylumbelliferyl-6-sulfo-2-acetamido-2-deoxy-beta-glucopyranoside
used for diagnosis of B variants of GM2 gangliosidosis
Also Known As:
4-methylumbelliferyl-beta-D-N-acetylglucosamine-6-sulfate; 4MUGS; Mu-GlcNAcS
Networked:
3
relevant articles (
0
outcomes,
0
trials/studies)
Bio-Agent Context: Research Results
Heterocyclic Compounds: 60
Pyrans: 5
Benzopyrans: 11
Coumarins: 240
Umbelliferones: 2
Hymecromone: 13
4-methylumbelliferyl-6-sulfo-2-acetamido-2-deoxy-beta-glucopyranoside: 3
2-Ring Heterocyclic Compounds
Benzopyrans: 11
Coumarins: 240
Umbelliferones: 2
Hymecromone: 13
4-methylumbelliferyl-6-sulfo-2-acetamido-2-deoxy-beta-glucopyranoside: 3
Related Diseases
1.
GM2 Gangliosidoses (GM2 Gangliosidosis)
11/15/1984 - "
Synthesis of 4-methylumbelliferyl-beta-D-N-acetylglucosamine-6-sulfate and its use in classification of GM2 gangliosidosis genotypes.
"
07/01/1993 - "
Here we report the observation of a German/Hungarian boy aged 12 when he died with a prolonged course of a neurodegenerative disorder, later biochemically identified as a GM2 gangliosidosis B1-variant which is characterized by a deficient Hex A activity only against 4MUGS.
"
07/01/1993 - "
After the introduction of 4-methylumbelliferyl-2-acetamido-2-deoxy-beta A-D-glucopyranoside (4MUG) and its sulfated form (4MUGS) in the pre- and postnatal diagnosis and carrier identification of gangliosidosis genotypes, infrequent forms of the GM2 gangliosidosis Type B (Tay-Sachs disease) have been observed which show normal activity of Hexosaminidase A (Hex A) isoenzyme with the substrate 4MUG but absent or deficient activity against the sulfated form 4MUGS.
"
2.
Tay-Sachs Disease
05/01/1989 - "
The patients had 0.32% and 0.36% of Hex A-associated 4MUGS cleaving activity compared to normal control fibroblasts as compared to less than 0.016% for infantile Tay-Sachs disease fibroblasts.
"
07/01/1993 - "
After the introduction of 4-methylumbelliferyl-2-acetamido-2-deoxy-beta A-D-glucopyranoside (4MUG) and its sulfated form (4MUGS) in the pre- and postnatal diagnosis and carrier identification of gangliosidosis genotypes, infrequent forms of the GM2 gangliosidosis Type B (Tay-Sachs disease) have been observed which show normal activity of Hexosaminidase A (Hex A) isoenzyme with the substrate 4MUG but absent or deficient activity against the sulfated form 4MUGS.
"
3.
Neurodegenerative Diseases (Neurodegenerative Disease)
07/01/1993 - "
Here we report the observation of a German/Hungarian boy aged 12 when he died with a prolonged course of a neurodegenerative disorder, later biochemically identified as a GM2 gangliosidosis B1-variant which is characterized by a deficient Hex A activity only against 4MUGS.
"
4.
Gangliosidoses (Gangliosidosis)
07/01/1993 - "
After the introduction of 4-methylumbelliferyl-2-acetamido-2-deoxy-beta A-D-glucopyranoside (4MUG) and its sulfated form (4MUGS) in the pre- and postnatal diagnosis and carrier identification of gangliosidosis genotypes, infrequent forms of the GM2 gangliosidosis Type B (Tay-Sachs disease) have been observed which show normal activity of Hexosaminidase A (Hex A) isoenzyme with the substrate 4MUG but absent or deficient activity against the sulfated form 4MUGS.
"
Related Drugs and Biologics
1.
Hexosaminidase A (Hex A)