title: Congenital disorders of amino acid metabolism
text: # Congenital disorders of amino acid metabolism
  
  Inborn errors of amino acid metabolism are metabolic disorders which impair the synthesis and degradation of amino acids.
  
  ## Types
  
  * Alkaptonuria
  * Aspartylglucosaminuria
  * Branched-chain keto acid dehydrogenase kinase deficiency
  * Methylmalonic acidemia
  * Maple syrup urine disease
  * Homocystinuria
  * Tyrosinemia
  * Trimethylaminuria
  * Hartnup disease
  * Biotinidase deficiency
  * Ornithine carbamoyltransferase deficiency
  * Carbamoyl-phosphate synthase I deficiency disease
  * Citrullinemia
  * Hyperargininemia
  * Hyperhomocysteinemia
  * Hypermethioninemia
  * Hyperlysinemias
  * Nonketotic hyperglycinemia
  * Propionic acidemia
  * Hyperprolinemia
  
  ## Amino acid transport disorders
  
  * Cystinuria
  * Dicarboxylic aminoaciduria
  * Hartnup disease
  
  ## Amino acid storage disorders
  
  * Glutaric acidemia type 2
  