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: 198
- 1-Ring Heterocyclic Compounds
  - Pyrans: 130
    - Benzopyrans: 195
      - Coumarins: 826
        Umbelliferones: 3
        Hymecromone: 123
        4-methylumbelliferyl-6-sulfo-2-acetamido-2-deoxy-beta-glucopyranoside: 3
- Fused-Ring Heterocyclic Compounds
  - 2-Ring Heterocyclic Compounds
    - Benzopyrans: 195
      - Coumarins: 826
        Umbelliferones: 3
        Hymecromone: 123
        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)
2.	Isoenzymes