Template:Amino acid metabolic pathology - WikiMD.com - Food, Medicine & Wellness Encyclopedia

Lysine/straight chain	* Glutaric acidemia type 1 type 2 Hyperlysinemia Pipecolic acidemia Saccharopinuria
Leucine	* 3-hydroxy-3-methylglutaryl-CoA lyase deficiency 3-Methylcrotonyl-CoA carboxylase deficiency 3-Methylglutaconic aciduria 1 Isovaleric acidemia Maple syrup urine disease
Tryptophan	* Hypertryptophanemia

Histidine	* Carnosinemia Histidinemia Urocanic aciduria
Proline	* Hyperprolinemia Prolidase deficiency
Glutamate/glutamine	* SSADHD

Valine	* Hypervalinemia Isobutyryl-CoA dehydrogenase deficiency Maple syrup urine disease
Isoleucine	* 2-Methylbutyryl-CoA dehydrogenase deficiency Beta-ketothiolase deficiency Maple syrup urine disease
Methionine	* Cystathioninuria Homocystinuria Hypermethioninemia
General BC/OA	* Methylmalonic acidemia Methylmalonyl-CoA mutase deficiency Propionic acidemia