<unk>  is an autosomal recessive metabolic disorder characterized by an increased urinary excretion of<unk><unk>  acid and<unk><unk>  acid. It is caused by mutations in DHTKD1, which encodes the E1 subunit of the oxoglutarate dehydrogenase complex<unk> -ketoglutarate dehydrogenase complex).
Alpha-aminoadipic and alpha-ketoadipic aciduria
Hawkinsinuria
Urocanic aciduria
Trimethylaminuria
3-Methylglutaconic aciduria
Orotic aciduria
Alkaptonuria
Homocystinuria