3-methylglutaconic aciduria https://www.malacards.org/card/3_methylglutaconic_aciduria OMIMPS:250950 MEDGEN:777186 ICD10CM:E71.111 NANDO:1200989 A group of five inherited disorders caused by mutations in the AUH, DNAJC19, OPA3, and TAZ genes. The disorders are characterized by impairment in the function of mitochondria, resulting in the accumulation and excretion of 3-methylglutaconic acid, and the presence of 3-methylglutaric acid in the urine. Orphanet:289902 MESH:C579867 DOID:0060336 SCTID:237950009 NANDO:2200496 icd11.foundation:1008261602 MONDO:0017359 NCIT:C98678 GARD:0012966 UMLS:C3696376 classic organic aciduria