FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term propionic acidemia ID (Ontology) DOID:14701 (Human Disease)
Definition An organic acidemia that involes a nonfunctional propionyl CoA carboxylase affecting conversion of amino acids and fats into sugar for energy.
Also Known As "GLYCINEMIA, KETOTIC" ; "ketotic glycinemia" ; "KETOTIC HYPERGLYCINEMIA" (for all, see Synonyms field below)
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 Genes
 propionic acidemia       1
 for disease ribbon | propionic acidemia       1
 model of | propionic acidemia       1
Spanning Tree (Parents/Children)
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  amino acid metabolic disorder
   |__organic acidemia
       |__propionic acidemia  1 rec.
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Is a organic acidemia
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Synonyms
  • "GLYCINEMIA, KETOTIC" EXACT
    "ketotic glycinemia" EXACT
    "KETOTIC HYPERGLYCINEMIA" EXACT
    "ketotic II glycinemia" EXACT
    "propionic aciduria" EXACT
    "propionyl-CoA carboxylase deficiency" EXACT
Secondary IDs
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GARD:467
ICD10CM:E71.121
MESH:D056693
MIM:606054
NCI:C85030
SNOMEDCT_US_2023_03_01:69080001
UMLS_CUI:C0268579
UMLS_CUI:C2717876