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Sandhoff Disease
(Sandhoff's Disease)
Summary
Description:
An autosomal recessive neurodegenerative disorder characterized by an accumulation of G(M2) GANGLIOSIDE in neurons and other tissues. It is caused by mutation in the common beta subunit of HEXOSAMINIDASE A and HEXOSAMINIDASE B. Thus this disease is also known as the O variant since both hexosaminidase A and B are missing. Clinically, it is indistinguishable from TAY-SACHS DISEASE.
Also Known As:
Sandhoff's Disease; Adult Sandhoff Disease; Deficiency Disease, Hexosaminidase A and B; GM2 Gangliosidosis, Type 2; GM2 Gangliosidosis, Type II Show All >>
Networked: 225
relevant articles (5 outcomes,
15 trials/studies)
for this Disease
Key Drugs and Agents for Sandhoff Disease
Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
-
N-butyldeoxygalactonojirimycin
:
1 outcome 1 study in 2 results
: IBA
-
Biological Markers (Surrogate Marker)
:
1 outcome in 1 result
: IBA
-
Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)
:
1 outcome in 1 result
: IBA
-
Liposomes (Liposome)
:
1 outcome in 1 result
: IBA
-
miglustat (Zavesca)
:
4 studies in 10 results
:
FDA 1
-
beta-N-Acetylhexosaminidases
:
2 studies in 66 results
: IBA
-
Hexosaminidase A (Hex A)
:
2 studies in 38 results
: IBA
-
First Step
:
2 studies in 2 results
: IBA
-
Hexosaminidases (Hexosaminidase)
:
1 study in 57 results
: IBA
-
G(M2) Ganglioside (Ganglioside GM2)
:
1 study in 43 results
: IBA
Show All >>
Diseases Related to Sandhoff Disease
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Lysosomal Storage Diseases (Lysosomal Storage Disease)
-
Neurologic Manifestations (Neurological Manifestations)
-
Disease Progression
-
Tay-Sachs Disease
-
GM2 Gangliosidoses (GM2 Gangliosidosis)
Show All >>
Key Therapies for Sandhoff Disease
Efficacy Chart >>
-
Bone Marrow Transplantation (Transplantation, Bone Marrow)
:
1 outcome in 3 results
-
Caloric Restriction
:
2 studies in 3 results
-
Transplantation (Transplant Recipients)
:
1 study in 3 results
-
Ketogenic Diet
:
2 results
-
Enzyme Replacement Therapy
:
1 result
Show All >>
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