DiseaseID 25765

溶酶体βA甘露糖苷贮积症

Mannosidosis, Beta A, Lysosomal

NCI2016_02D:An autosomal recessive lysosomal storage disease characterized by a deficient activity of the enzyme beta-mannosidase. It is caused by mutations in the MANBA gene. Common features of this disorder are mental

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Disease: 1Symptom: 7Target: 17Links: 31
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Record Fields

Scalar fields from the final disease record.

Disease Id
25765
Core Entity Id
118333
Source Entity Count
1
Preferred Name
Mannosidosis, Beta A, Lysosomal
Name Cn
溶酶体βA甘露糖苷贮积症
Name Pinyin
Rong Mei Ti Βa Gan Lu Tang Gan Zhu Ji Zheng
Name En
Mannosidosis, Beta A, Lysosomal
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
NCI2016_02D:An autosomal recessive lysosomal storage disease characterized by a deficient activity of the enzyme beta-mannosidase. It is caused by mutations in the MANBA gene. Common features of this disorder are mental retardation, developmental delays and recurrent respiratory infections.|MSH2017_2016_08_12:An inborn error of metabolism marked by a defect in the lysosomal isoform of BETA-MANNOSIDASE that results in lysosomal accumulation of mannose-rich intermediate metabolites containing 1,4-beta linkages. The human disease occurs through autosomal recessive inheritance and manifests in the form of a variety of symptoms that depend upon the type of gene mutation.
Version
v1,v2
Suppressed
No

Names

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

Name
Mannosidosis, Beta A, Lysosomal
Role
preferred

Cross References

Trusted external identifiers retained for this final record.

Umls
C4048196
Sym Map
SMDE00251
Etcm Disease
Mannosidosis, Beta a, Lysosomal
Itcmdb Generated
ITX-DISEASE-E0B18D6371A3

Attributes

Merged source attributes and domain-specific metadata.

Version
v1,v2
Suppress
0
Page Title
Disease Mannosidosis, Beta a, Lysosomal Details page
Basic Information
Disease Name
Mannosidosis, Beta a, Lysosomal
Global Category
Genetic diseases;Metabolic diseases;Rare diseases
Anatomical Category
Bone diseases;Eye diseases;Mental diseases;Neuronal diseases
Disease Definition
NCI2016_02D:An autosomal recessive lysosomal storage disease characterized by a deficient activity of the enzyme beta-mannosidase. It is caused by mutations in the MANBA gene. Common features of this disorder are mental retardation, developmental delays and recurrent respiratory infections.|MSH2017_2016_08_12:An inborn error of metabolism marked by a defect in the lysosomal isoform of BETA-MANNOSIDASE that results in lysosomal accumulation of mannose-rich intermediate metabolites containing 1,4-beta linkages. The human disease occurs through autosomal recessive inheritance and manifests in the form of a variety of symptoms that depend upon the type of gene mutation.