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Sodium-dependent neutral amino acid transporter B(0)AT1 (SLC6A19 (also known as B0AT1))

Target
SLC6A19 (also known as B0AT1)
Molecular classification
Transporter, Solute carrier family (SLC), Sodium-dependent transporter
01

Overview

Sodium-dependent neutral amino acid transporter B(0)AT1 is a membrane protein encoded by the SLC6A19 gene. It mediates the sodium-dependent uptake and epithelial resorption of all neutral amino acids across the apical membrane in both kidney and intestinal epithelial cells. This function is critical for maintaining systemic levels of essential and non-essential neutral amino acids. For proper surface expression and catalytic activity, B(0)AT1 requires coexpression with collectrin (TMEM27). Mutations in this gene cause Hartnup disease, a disorder characterized by defective transport of certain amino acids leading to various clinical symptoms. The transporter has also been identified as a possible therapeutic target for metabolic diseases such as type 2 diabetes due to its central role in nutrient handling. Benztropine has been shown experimentally to act as a selective competitive inhibitor of this transporter.

Other names
SLC6A19B0AT1HNDsolute carrier family 6 member 19
02

Mechanism of action

Competitive inhibition of sodium-dependent uptake of neutral amino acids by blocking the transporter’s substrate binding site

03

Biological functions

Epithelial resorption of neutral amino acids in the kidney and intestineAmino acid transmembrane transport
04

Disease associations

Hartnup disease (caused by mutations in SLC6A19)Potential target for type 2 diabetes therapy
05

Safety considerations

Disruption can lead to impaired absorption of essential amino acids and metabolic disorders such as Hartnup disease; potential off-target effects if used therapeutically due to its role in nutrient absorption
06

Interacting drugs

Benztropine
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Biomarkers

Mutations in SLC6A19 gene serve as biomarkers for Hartnup disease diagnosis

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