Tay-Sachs Disease, AB Variant [Tay-Sachsova nemoc, varianta AB]

topical
Terms

aktivátor proteinu GM2 - nedostatek
deficit G(M2) aktivátorového proteinu
gangliosidóza GM2 - typ AB
gangliosidóza GM2 - varianta AB
nedostatek G(M2) aktivátorového proteinu
Tayova-Sachsova nemoc, typ AB

 

AB Variant Gangliosidosis GM2
AB Variant GM2-Gangliosidosis
Activator Deficiency GM2 Gangliosidosis
Activator-Deficient Tay-Sachs Disease
Deficiency Disease, GM2 Protein Activator
Gangliosidosis GM2, AB Variant
Gangliosidosis GM2, Type AB
GM2 Activator Deficiency
GM2 Activator Deficiency Disease
GM2 Gangliosidosis, Type AB
GM2 Protein Activator Deficiency Disease
GM2-Gangliosidosis, AB Variant
Hexosaminidase Activator Deficiency
Hexosaminidase Activator Protein Deficiency Disease

Persistent link   https://www.medvik.cz/link/D049290
Definition

A progressive neurodegenerative disorder that begins with muscle weakness, then progresses to startle reaction, retardation and seizures. It is characterized by the accumulation of G(M2) GANGLIOSIDE in neurons that is caused by a lack of G(M2) ACTIVATOR PROTEIN function. The AB variant designation refers to the increase of both HEXOSAMINIDASE A and HEXOSAMINIDASE B in tissues that lack of G(M2) activator protein.

DUI
D049290 MeSH Browser
CUI
M0335225
Previous indexing
Gangliosidoses (2000-2004)
History note
2005(2000); use GANGLIOSIDOSES GM2 2000-2004
Public note
2005; see GANGLIOSIDOSES GM2 2000-2004

C Diseases
C10.228.140 Brain Diseases 1 183
C10.228.140.163 Brain Diseases, Metabolic 85
C10.228.140.163.100.435.825 Sphingolipidoses 12
C10.228.140.163.100.435.825.300 Gangliosidoses 11
C10.228.140.163.100.435.825.300.300 Gangliosidoses, GM2 2
C10.228.140.163.100.435.825.300.300.249 Sandhoff Disease 1
C10.228.140.163.100.435.825.300.300.500 Tay-Sachs Disease 15
C10.228.140.163.100.435.825.300.300.750 Tay-Sachs Disease, AB Variant
C16.320.565.189.435.825 Sphingolipidoses 12
C16.320.565.189.435.825.300 Gangliosidoses 11
C16.320.565.189.435.825.300.300 Gangliosidoses, GM2 2
C16.320.565.189.435.825.300.300.249 Sandhoff Disease 1
C16.320.565.189.435.825.300.300.500 Tay-Sachs Disease 15
C16.320.565.189.435.825.300.300.750 Tay-Sachs Disease, AB Variant
C16.320.565.398.641 Lipidoses 35
C16.320.565.398.641.803 Sphingolipidoses 12
C16.320.565.398.641.803.350 Gangliosidoses 11
C16.320.565.398.641.803.350.300 Gangliosidoses, GM2 2
C16.320.565.398.641.803.350.300.700 Sandhoff Disease 1
C16.320.565.398.641.803.350.300.850 Tay-Sachs Disease 15
C16.320.565.398.641.803.350.300.925 Tay-Sachs Disease, AB Variant
C16.320.565.595 Lysosomal Storage Diseases 73
C16.320.565.595.554.825 Sphingolipidoses 12
C16.320.565.595.554.825.300 Gangliosidoses 11
C16.320.565.595.554.825.300.300 Gangliosidoses, GM2 2
C16.320.565.595.554.825.300.300.800 Sandhoff Disease 1
C16.320.565.595.554.825.300.300.840 Tay-Sachs Disease 15
C16.320.565.595.554.825.300.300.920 Tay-Sachs Disease, AB Variant
C18.452 Metabolic Diseases 1 200
C18.452.132.100.435.825 Sphingolipidoses 12
C18.452.132.100.435.825.300 Gangliosidoses 11
C18.452.132.100.435.825.300.300 Gangliosidoses, GM2 2
C18.452.132.100.435.825.300.300.249 Sandhoff Disease 1
C18.452.132.100.435.825.300.300.500 Tay-Sachs Disease 15
C18.452.132.100.435.825.300.300.750 Tay-Sachs Disease, AB Variant
C18.452.584.563.641 Lipidoses 35
C18.452.584.563.641.803 Sphingolipidoses 12
C18.452.584.563.641.803.350 Gangliosidoses 11
C18.452.584.563.641.803.350.300 Gangliosidoses, GM2 2
C18.452.584.563.641.803.350.300.700 Sandhoff Disease 1
C18.452.584.563.641.803.350.300.850 Tay-Sachs Disease 15
C18.452.584.563.641.803.350.300.925 Tay-Sachs Disease, AB Variant
C18.452.648.189.435.825 Sphingolipidoses 12
C18.452.648.189.435.825.300 Gangliosidoses 11
C18.452.648.189.435.825.300.300 Gangliosidoses, GM2 2
C18.452.648.189.435.825.300.300.249 Sandhoff Disease 1
C18.452.648.189.435.825.300.300.500 Tay-Sachs Disease 15
C18.452.648.189.435.825.300.300.750 Tay-Sachs Disease, AB Variant
C18.452.648.398.641 Lipidoses 35
C18.452.648.398.641.803 Sphingolipidoses 12
C18.452.648.398.641.803.350 Gangliosidoses 11
C18.452.648.398.641.803.350.300 Gangliosidoses, GM2 2
C18.452.648.398.641.803.350.300.700 Sandhoff Disease 1
C18.452.648.398.641.803.350.300.850 Tay-Sachs Disease 15
C18.452.648.398.641.803.350.300.925 Tay-Sachs Disease, AB Variant
C18.452.648.595 Lysosomal Storage Diseases 73
C18.452.648.595.554.825 Sphingolipidoses 12
C18.452.648.595.554.825.300 Gangliosidoses 11
C18.452.648.595.554.825.300.300 Gangliosidoses, GM2 2
C18.452.648.595.554.825.300.300.800 Sandhoff Disease 1
C18.452.648.595.554.825.300.300.840 Tay-Sachs Disease 15
C18.452.648.595.554.825.300.300.920 Tay-Sachs Disease, AB Variant