JX237 functions as an inhibitor targeting the epithelial neutral amino acid transporter B 0 AT1 (SLC6A19), exhibiting a potency with an IC 50 of 31 nM. This compound primarily facilitates the absorption of neutral amino acids in the intestines and their reabsorption in the kidneys. The inhibition of B 0 AT1 by JX237 is instrumental in normalizing high levels of plasma amino acids found in rare metabolic amino acid disorders such as phenylketonuria and urea cycle disorders.