DiseaseID 7521

嘌呤核苷磷酸化酶缺乏症

disease

NCI2016_02D:PNP reversibly catalyzes the phosphorolysis of the purine nucleosides, (deoxy)inosine and (deoxy)guanosine, to their respective purine bases and the corresponding ribose-1-phosphate. Deficiency in this enzyme

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Disease: 1Symptom: 4Target: 2Links: 6
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Record Fields

Scalar fields from the final disease record.

Disease Id
7521
Core Entity Id
64323
Source Entity Count
1
Preferred Name
Purine-Nucleoside Phosphorylase Deficiency
Name Cn
嘌呤核苷磷酸化酶缺乏症
Name Pinyin
Piao Ling He Gan Lin Suan Hua Mei Que Fa Zheng
Name En
Purine-Nucleoside Phosphorylase Deficiency
Name Latin
Bilingual Status
complete
Disease Type
disease
Umls Disease Type
Disease or Syndrome
Disgenet Type
disease
Mesh Class
Immune System Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
Do Class
disease of anatomical entity; genetic disease
Hpo Class
Mesh Class Name
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Immune System Diseases
Hpo Class Name
Do Class Name
genetic disease; disease of anatomical entity
Disease Definition
NCI2016_02D:PNP reversibly catalyzes the phosphorolysis of the purine nucleosides, (deoxy)inosine and (deoxy)guanosine, to their respective purine bases and the corresponding ribose-1-phosphate. Deficiency in this enzyme is an autosomal recessive cause of combined immunodeficiency.
Version
v1,v2
Suppressed
No

Names

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

Name
Purine-Nucleoside Phosphorylase Deficiency
Role
preferred
Name
Purine Nucleoside Phosphorylase Deficiency
Role
preferred
Name
NUCLEOSIDE PHOSPHORYLASE DEFICIENCY
Role
alias
Name
Purine Nucleoside Phosphorylase [Pnp] Deficiency
Role
alias

Cross References

Trusted external identifiers retained for this final record.

Herb
HBDIS006186
Omim
613179
Umls
C0268125
Icd10
D81.5
Sym Map
SMDE03474
Do Class
DOID:630DOID:7
Dis Ge Net
C0268125
Umls Sty
T047
Me Sh Class
C16C18C20
Tcmbank Disease
30711

Attributes

Merged source attributes and domain-specific metadata.

Version
v1,v2
Suppress
0
Do Class Name
genetic disease; disease of anatomical entity
Disease Type
disease
Do Disease Class
disease of anatomical entity; genetic disease
Umls Disease Type
Disease or Syndrome
Disease Definition
NCI2016_02D:PNP reversibly catalyzes the phosphorolysis of the purine nucleosides, (deoxy)inosine and (deoxy)guanosine, to their respective purine bases and the corresponding ribose-1-phosphate. Deficiency in this enzyme is an autosomal recessive cause of combined immunodeficiency.
Me Sh Disease Class
Immune System Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Immune System Diseases
Umls Semantic Type Name
Disease or Syndrome