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Descriptor English: Gangliosidoses
Descriptor Spanish: Gangliosidosis
Descriptor gangliosidosis
Scope note: Grupo de enfermedades hereditarias, a menudo fatales, que se caracterizan por acumulación de GANGLIÓSIDOS en los LISOSOMAS, secundaria a estados de deficiencia enzimática. Las gangliosidosis incluyen la ENFERMEDAD DE TAY-SACHS, GANGLIOSIDOSIS GM1, GANGLIOSIDOSIS GM2 y la ENFERMEDAD DE SANDHOFF, que comparten el comienzo neonatal o infantil del deterioro del SISTEMA NERVIOSO CENTRAL. (Menkes, Textbook of Child Neurology, 5th ed, pp89-97)
Descriptor Portuguese: Gangliosidoses
Descriptor French: Gangliosidoses
Entry term(s): Ganglioside Storage Disease
Ganglioside Storage Diseases
Ganglioside Storage Disorder
Ganglioside Storage Disorders
Gangliosidosis
Storage Disease, Ganglioside
Storage Diseases, Ganglioside
Storage Disorder, Ganglioside
Storage Disorders, Ganglioside
Tree number(s): C10.228.140.163.100.435.825.300
C16.320.565.189.435.825.300
C16.320.565.398.641.803.350
C16.320.565.595.554.825.300
C18.452.132.100.435.825.300
C18.452.584.687.803.350
C18.452.648.189.435.825.300
C18.452.648.398.641.803.350
C18.452.648.595.554.825.300
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D005733
Scope note: 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.
Annotation: coordinate IM with specific ganglioside (IM) but GANGLIOSIDOSIS, GM1 and GANGLIOSIDOSES, GM2 are available
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Gangliosides (1966-1975)
Lipoidosis (1966-1975)
Public MeSH Note: 1992; see GANGLIOSIDOSIS 1976-91
History Note: 1992(1976)
Related: Mucolipidoses MeSH
DeCS ID: 30336
Unique ID: D005733
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1976/01/01
Date of Entry: 1975/07/23
Revision Date: 2010/06/25
Gangliosidoses - Preferred
Concept UI M0008988
Scope note 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.
Preferred term Gangliosidoses
Entry term(s) Ganglioside Storage Disease
Ganglioside Storage Diseases
Ganglioside Storage Disorder
Ganglioside Storage Disorders
Gangliosidosis
Storage Disease, Ganglioside
Storage Diseases, Ganglioside
Storage Disorder, Ganglioside
Storage Disorders, Ganglioside



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