Introduction Pocket Metab Impressum
··· Diseases ··· Symptoms ··· Lab Parameters ··· Authors ··· Ramedis ··· BH4 Patient List of associated diseases and any hint to further information based on the descripton or synonyms to the accession number HMDB00208
Oxoglutaric acid
Alpha-ketoglutaric acid is an important biological compound and is a key intermediate in the Krebs cycle. Alpha-ketoglutaric acid occurs naturally within cells. One of its functions is to combine with ammonia to form glutamic acid and then glutamine. Another function is to combine with nitrogen released in the cell, therefore preventing nitrogen overload. (wikipedia)
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  1. 2-KETOGLUTARATE DEHYDROGENASE COMPLEX DEFICIENCY
  2. GLUTARIC ACIDURIA I
  3. GLUTARIC ACIDURIA II
  4. 3-METHYL-CROTONYL-GLYCINURIA
  5. FUMARIC ACIDURIA
  6. PYRUVATE DEHYDROGENASE DEFICIENCY (E3)
  7. LYSINURIC PROTEIN INTOLERANCE (LPI)
  8. 2-HYDROXYGLUTARIC ACIDURIA (D)
  9. PYRUVATE CARBOXYLASE DEFICIENCY
  10. REFSUM DISEASE
  11. NEWBORNS, PREMATURE INFANTS, TRANSIENT IMMATURITY OF TRANSPORT SYSTEMS OR ENZYMES
  12. PRIMARY HYPEROXALURIA I, PH1
  13. MUCOLIPIDOSIS II
  14. 3-METHYLGLUTACONIC ACIDURIA (TYPE IV)
  15. ARTEFACTS-BACTERIAL CONTAMINATION, EXCRETION OF ORGANIC ACIDS (ARTEFACTS) [DD]
  16. RETT SYNDROME
  17. HYPERINSULINISM-HYPERAMMONEMIA SYNDROME