Sodium-dependent neutral amino acid transporter B(0)AT1 (SLC6A19) (SLC6A19)

Sodium-dependent neutral amino acid transporter B(0)AT1 (SLC6A19) is the primary apical transporter for neutral amino acids in the small intestine and the renal proximal tubule (UniProt: Q695T7). It operates as a symporter, utilizing the electrochemical gradient of sodium to drive the uptake of essential amino acids, including tryptophan and branched-chain amino acids (PubMed: 15286787). The functional expression of B(0)AT1 is dependent on its association with accessory proteins like ACE2 in the gut and collectrin in the kidney (PubMed: 18424768). Mutations in the SLC6A19 gene result in Hartnup disease, a condition marked by impaired amino acid absorption and symptoms resembling pellagra due to tryptophan deficiency (NIH: Hartnup Disease). In modern pharmacology, SLC6A19 is being explored as a target for metabolic disorders; inhibiting the transporter can reduce systemic amino acid levels, which may improve glucose tolerance and support weight loss (PubMed: 30115614). Furthermore, blocking B(0)AT1-mediated phenylalanine absorption offers a potential therapeutic avenue for managing phenylketonuria (PubMed: 29453437).

Other names

B0AT1System B(0) neutral amino acid transporterSolute carrier family 6 member 19Hartnup disorder proteinB0 system transporter

Inhibition of sodium-dependent neutral amino acid transport in the intestinal brush border and renal proximal tubule