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Gangliosidoses (Gangliosidosis)

A group of autosomal recessive lysosomal storage disorders marked by the accumulation of GANGLIOSIDES. They are caused by impaired enzymes or defective cofactors required for normal ganglioside degradation in the LYSOSOMES. Gangliosidoses are classified by the specific ganglioside accumulated in the defective degradation pathway.
Also Known As:
Gangliosidosis; Ganglioside Storage Diseases; Ganglioside Storage Disorders; Ganglioside Storage Disease; Ganglioside Storage Disorder; Storage Disease, Ganglioside; Storage Diseases, Ganglioside; Storage Disorder, Ganglioside; Storage Disorders, Ganglioside
Networked: 86 relevant articles (1 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Mucopolysaccharidoses
2. Tay-Sachs Disease
3. GM1 Gangliosidosis (Gangliosidosis GM1)
4. Mucopolysaccharidosis IV (Morquio Syndrome)
5. Fabry Disease (Fabry's Disease)

Experts

1. Seyfried, Thomas N: 4 articles (09/2015 - 04/2007)
2. Platt, Frances M: 4 articles (06/2009 - 08/2003)
3. Jeyakumar, Mylvaganam: 4 articles (06/2009 - 08/2003)
4. Butters, Terry D: 3 articles (04/2010 - 08/2004)
5. Dwek, Raymond A: 3 articles (04/2010 - 08/2004)
6. Akgoc, Zeynep: 2 articles (09/2015 - 05/2015)
7. d'Azzo, Alessandra: 2 articles (05/2015 - 06/2007)
8. Martin, Douglas R: 2 articles (05/2015 - 05/2004)
9. Smith, David: 2 articles (06/2009 - 08/2004)
10. Futerman, Anthony H: 2 articles (01/2008 - 08/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Gangliosidoses:
1. beta-Galactosidase (Lactaid)IBA
2. GangliosidesIBA
3. Hexosaminidases (Hexosaminidase)IBA
4. Neutral GlycosphingolipidsIBA
5. Fluorescein (Funduscein)FDA LinkGeneric
6. Protein Kinase CIBA
7. Galactose (Galactopyranose)FDA LinkGeneric
8. Carisoprodol (Soma)FDA LinkGeneric
9. Amino AcidsFDA Link
10. 1-deoxygalactonojirimycinIBA

Therapies and Procedures

1. Deep Brain Stimulation
2. Transplantation (Transplant Recipients)
3. Euthanasia (Mercy Killing)