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 HMDB00522
3-Methylglutaconic acid
3-methylglutaconyl-CoA hydratase is involved in the metabolism process of 3-methylglutaconic acid. 3-Methylglutaconic acid accumulates in patients with a deficiency of this enzyme and a large amount of 3-methylglutaconic acid appear in urine. (Wikipedia)
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  1. 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
  2. BETA-MANNOSIDOSIS
  3. 3-METHYLGLUTACONIC ACIDURIA (TYPE I)
  4. 3-METHYLGLUTACONIC ACIDURIA (TYPE II), X-LINKED
  5. LETHAL INFANTILE CARDIOMYOPATHY: X-LINKED CARDIOSKELETAL MYOPATHY (BARTH SYNDROME)
  6. 3-METHYLGLUTACONIC ACIDURIA (TYPE IV)
  7. PEARSON SYNDROM
  8. SMITH-LEMLI-OPITZ SYNDROME
  9. CREATINE DEFICIENCY, GUANIDINOACETATE METHYLTRANSFERASE DEFICIENCY
  10. 3-METHYLGLUTACONIC ACIDURIA (TYPE III)