Basic transport of glutamate in the synapse as well as the neuroglia. Glutamate transporters are proficient at pumping glutamate into cells due to their ability to couple with inorganic ions. Transport of glutamate into the cell requires the coupling of three sodium ions as well as a proton, whereas transport out of the cell requires a single potassium ion. This transport results in two positive charges being displaced across the membrane per cycle. Moreover, this process is dependent on a pH gradient, due to glutamate needing to be protenated prior to transport. Mutations<unk>  is the result of a point mutation of tryptophan to arginine at position 445 and a deletion mutation of isoleucine at position 395. EAAT3 is found in location 9p24, it is primarily expressed in the brain and kidneys. Metabolism In the gastrointestinal tract, protein digestion and absorption are key to establishing and maintaining amino acid pools. In the case of<unk> , where glutamate and aspartate transport are impaired, the alanine, aspartate and glutamate metabolism are affected. Enterocytes in the intestines break up peptides into residual amino acids where they would normally use charge-specific amino acid transporters to get across the epithelial cells. In<unk> , the anionic amino acid transporter, EAAT3, cannot bring glutamate and aspartate across epithelial cells, leading to them being excreted via the urine. Examples Below is an example of how glutamate is used to synthesize alanine via alanine transaminase. :Glutamate + pyruvate ⇌ α-ketoglutarate + Alanine Alanine transaminase Another example is the conversion of aspartate to glutamate via the enzyme aspartate transaminase. :Aspartate + α-ketoglutarate ⇌ Oxaloacetate + Glutamate Aspartate transaminase.
Dicarboxylic aminoaciduria
Hawkinsinuria
Aspartylglucosaminuria
Essential fructosuria
Alkaptonuria
Homocystinuria
Alpha-aminoadipic and alpha-ketoadipic aciduria
Urocanic aciduria