DiseaseID 26327

GM1神经节苷脂贮积症III型

Gm1-Gangliosidosis, Type Iii

JABL99:A ganglioside storage disorder caused by beta-galactosidase (EC 3.2.1.23) deficiency with resulting accumulation of excessive amounts of ganglioside GM1 in the brain tissue with less severe abnormalities than thos

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Disease: 1Symptom: 2Target: 18Links: 26
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Record Fields

Scalar fields from the final disease record.

Disease Id
26327
Core Entity Id
118895
Source Entity Count
1
Preferred Name
Gm1-Gangliosidosis, Type Iii
Name Cn
GM1神经节苷脂贮积症III型
Name Pinyin
Gm1 Shen Jing Jie Gan Zhi Zhu Ji Zheng Iii Xing
Name En
Gm1-Gangliosidosis, Type Iii
Name Latin
Bilingual Status
complete
Disease Type
Umls Disease Type
Disgenet Type
Mesh Class
Do Class
Hpo Class
Mesh Class Name
Hpo Class Name
Do Class Name
Disease Definition
JABL99:A ganglioside storage disorder caused by beta-galactosidase (EC 3.2.1.23) deficiency with resulting accumulation of excessive amounts of ganglioside GM1 in the brain tissue with less severe abnormalities than those seen in other gangliosidoses and inconsistent phenotype. Onset takes place in late teens or early adult life with cerebellar dysfunction, visual problems, and coarsening of facial features. Intellectual impairment is mild at first but intelligence tends to deteriorate in time.
Version
v1
Suppressed
No

Names

Preferred names, aliases, and source labels retained in the final schema.

Name
Gm1-Gangliosidosis, Type Iii
Role
preferred

Cross References

Trusted external identifiers retained for this final record.

Umls
C0268273
Sym Map
SMDE02974
Etcm Disease
Gm1-Gangliosidosis, Type Iii
Itcmdb Generated
ITX-DISEASE-00A0036C98FE

Attributes

Merged source attributes and domain-specific metadata.

Version
v1
Suppress
0
Page Title
Disease Gm1-Gangliosidosis, Type Iii Details page
Basic Information
Disease Name
Gm1-Gangliosidosis, Type Iii
Global Category
Genetic diseases;Metabolic diseases;Rare diseases
Anatomical Category
Bone diseases;Eye diseases;Liver diseases;Neuronal diseases
Disease Definition
JABL99:A ganglioside storage disorder caused by beta-galactosidase (EC 3.2.1.23) deficiency with resulting accumulation of excessive amounts of ganglioside GM1 in the brain tissue with less severe abnormalities than those seen in other gangliosidoses and inconsistent phenotype. Onset takes place in late teens or early adult life with cerebellar dysfunction, visual problems, and coarsening of facial features. Intellectual impairment is mild at first but intelligence tends to deteriorate in time.