adisease

Showing entry for Intermediate Maple Syrup Urine Disease

　　　　　　　　　　　　　　　　

General Disease Information
BXGD Id	BXGD013467
Disease Name	Intermediate Maple Syrup Urine Disease
Disease CUI Id	C1621920
MeSH Codes	C16 C18 C10
Disease Class Name	Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Nervous System Diseases
Semantic Type	Disease or Syndrome
Human Phenotype Ontology Id
Human Phenotype Ontology Term
Disease Ontology Id	DOID:0014667 DOID:630
Disease Ontology Class Name	disease of metabolism; genetic disease
Disorder Network	disorder-protein-compound-food associations

The disease-related target proteins

Proteins

Uniport Id	BXGT Id	Protein Name	Gene	Status	Protein Class
Uniport Id	BXGT Id	Protein name	Gene	Status	Protein class
O15382	BXGT004160	Branched-chain-amino-acid aminotransferase, mitochondrial	587	reviewed	Enzyme
P12694	BXGT007835	2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial	593	reviewed
P21953	BXGT008711	2-oxoisovalerate dehydrogenase subunit beta, mitochondrial	594	reviewed	Enzyme
Q8N3J5	BXGT018506	Protein phosphatase 1K, mitochondrial	152926	reviewed	Enzyme

The disease-related compounds

Compounds

BXGC Id	Compound Name	Structure	Molecular Weight	Number of targets^{?Number of times compound connecting the disease , higher numbers indicate the compound is more associated with the disease}

The disease-related foods

Foods

BXGF Id	Food Name	Scientific Name	Food Group	Number of compounds in food^{?Number of compounds in food that connecting disease, higher numbers indicate the more compounds in food are more associated with the dusease}