Phenylalanine sensitive K562-D cells for the analysis of the biochemical impact of excess amino acid.

ABSTRACT: Although it is recognized that the abnormal accumulation of amino acid is a cause of the symptoms in metabolic disease such as phenylketonuria (PKU), the relationship between disease severity and serum amino acid levels is not well understood due to the lack of experimental model. Here, we present a novel in vitro cellular model using K562-D cells that proliferate slowly in the presence of excessive amount of phenylalanine within the clinically observed range, but not phenylpyruvate. The increased expression of the L-type amino acid transporter (LAT2) and its adapter protein 4F2 heavy chain appeared to be responsible for the higher sensitivity to phenylalanine in K562-D cells. Supplementation with valine over phenylalanine effectively restored cell proliferation, although other amino acids

SUBMITTER: Sanayama Y 

PROVIDER: S-EPMC4221789 | biostudies-literature | 2014 Nov

REPOSITORIES: biostudies-literature