Search Login Register

Tay-Sachs Disease Summary

Description: An autosomal recessive neurodegenerative disorder characterized by the onset in infancy of an exaggerated startle response, followed by paralysis, dementia, and blindness. It is caused by mutation in the alpha subunit of the HEXOSAMINIDASE A resulting in lipid-laden ganglion cells. It is also known as the B variant (with increased HEXOSAMINIDASE B but absence of hexosaminidase A) and is strongly associated with Ashkenazic Jewish ancestry.

Also Known As: Amaurotic Familial Idiocy; B Variant GM2 Gangliosidosis; Deficiency Disease Hexosaminidase A; Familial Amaurotic Idiocy; GM2 Gangliosidosis, B Variant Show All >>

Networked: 368 relevant articles (2 outcomes, 21 trials/studies) for this Disease

Key Drugs and Agents for Tay-Sachs Disease

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Hexosaminidase A (Hex A) : 1 outcome 4 studies in 122 results : IBA
  2. beta-N-Acetylhexosaminidases : 1 outcome 1 study in 95 results : IBA
  3. Lipids : 6 studies in 10 results : IBA
  4. Hexosaminidases (Hexosaminidase) : 2 studies in 44 results : IBA
  5. G(M2) Ganglioside (Ganglioside GM2) : 1 study in 30 results : IBA
  6. Enzymes : 1 study in 7 results : IBA
  7. RNA (Ribonucleic Acid) : 1 study in 3 results : IBA
  8. Proteins (Proteins, Gene) : 1 study in 2 results : IBA
  9. miglustat (Zavesca) : 1 study in 2 results : FDA 1
  10. Synucleins : 1 study in 1 result : IBA
Show All >>

Diseases Related to Tay-Sachs Disease

  1. Lipidoses
  2. Sandhoff Disease (Sandhoff's Disease)
  3. Metachromatic Leukodystrophy (Sulfatide Lipidosis)
  4. Gangliosidoses (Gangliosidosis)
  5. Adrenoleukodystrophy (Adrenoleukodystrophy, X-Linked)
Show All >>

Key Therapies for Tay-Sachs Disease

Efficacy Chart >>
  1. Enzyme Replacement Therapy : 2 results
  2. Transplants (Transplant) : 1 result
  3. Tissue Transplantation : 1 result
  4. Plasmapheresis : 1 result
  5. Lasers (Laser) : 1 result
Show All >>

CureHunter Inc. provides medical information and specifically does NOT provide medical advice.
© Copyright 2003-2016 CureHunter Inc., MeSH copyright NLM, Journal Articles copyright original owners.