page_title: Dicarboxylic aminoaciduria
section_title: Examples
hierarchical_section_title: Dicarboxylic aminoaciduria / Cause / Metabolism / Examples
caption_attribution_description: Français&#160;: Réaction catalysée par l'ALAT&#160;: Glu + Pyruvate ----&gt; acide α-cétoglutarique + Ala Création personnelle avec Chemtool et Sodipodi (février 2006).
context_page_description: Dicarboxylic aminoaciduria is a rare form of aminoaciduria which is an autosomal recessive disorder of urinary glutamate and aspartate due to genetic errors related to transport of these amino acids. Mutations resulting in a lack of expression of the SLC1A1 gene, a member of the solute carrier family, are found to cause development of dicarboxylic aminoaciduria in humans. SLC1A1 encodes for EAAT3 which is found in the neurons, intestine, kidney, lung, and heart. EAAT3 is part of a family of high affinity glutamate transporters which transport both glutamate and aspartate across the plasma membrane.
context_section_description: Below is an example of how glutamate is used to synthesize alanine via alanine transaminase.
  Glutamate + pyruvate ⇌  α-ketoglutarate + Alanine
  Another example is the conversion of aspartate to glutamate via the enzyme aspartate transaminase.
  Aspartate + α-ketoglutarate ⇌ Oxaloacetate + Glutamate
  .
caption: Alanine transaminase
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