- Concept UI
- M0335225
- Scope Note
- 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.
- Terms
-
Tay-Sachs Disease, AB Variant
Preferred Term
Term UI
T582131
Date04/08/2004
LexicalTag
EPO
ThesaurusID
-
Hexosaminidase Activator Deficiency
Term UI
T812202
Date11/15/2011
LexicalTag
NON
ThesaurusID
-
AB Variant GM2-Gangliosidosis
Term UI
T825150
Date06/27/2012
LexicalTag
ABX
ThesaurusID
OMIM (2013)
-
GM2 Activator Deficiency
Term UI
T825151
Date06/27/2012
LexicalTag
ABX
ThesaurusID
OMIM (2013)
-
GM2-Gangliosidosis, AB Variant
Term UI
T825152
Date06/27/2012
LexicalTag
ABX
ThesaurusID
-
Activator-Deficient Tay-Sachs Disease
Term UI
T841539
Date04/18/2013
LexicalTag
NON
ThesaurusID
NLM (2014)
-
GM2 Gangliosidosis, Type AB
Term UI
T843574
Date05/03/2013
LexicalTag
NON
ThesaurusID
GHR (2014)
-
Activator Deficiency GM2 Gangliosidosis
Term UI
T843575
Date05/03/2013
LexicalTag
NON
ThesaurusID
GHR (2014)
-
Deficiency Disease, GM2 Protein Activator
Term UI
T368288
Date10/12/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
Gangliosidosis GM2, AB Variant
Term UI
T368289
Date10/12/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
Gangliosidosis GM2, Type AB
Term UI
T368290
Date10/12/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
GM2 Activator Deficiency Disease
Term UI
T368292
Date10/12/1999
LexicalTag
NON
ThesaurusID
-
GM2 Protein Activator Deficiency Disease
Term UI
T368286
Date10/12/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
Hexosaminidase Activator Protein Deficiency Disease
Term UI
T368295
Date10/12/1999
LexicalTag
NON
ThesaurusID
NLM (2000)
-
AB Variant Gangliosidosis GM2
Term UI
T368287
Date10/12/1999
LexicalTag
NON
ThesaurusID
NLM (2000)