Large neutral amino acids auto exchange when infused by microdialysis into the rat brain: implication for maple syrup urine disease and phenylketonuria
HR Zielke, CL Zielke, PJ Baab, RM Collins - Neurochemistry international, 2002 - Elsevier
Maple syrup urine disease (MSUD) and phenylketonuria (PKU) are associated with
accumulation of large neutral amino acids (LNAA) in blood and tissues and a decrease of
other LNAA not directly related to the enzyme defects. One characteristic shared by both the
elevated and decreased amino acids is that all are substrates for transport via the large
neutral amino acid transporter. In this study, the blood brain barrier was effectively bypassed
using microdialysis to determine the immediate effect of infused phenylalanine, tyrosine, 2 …
accumulation of large neutral amino acids (LNAA) in blood and tissues and a decrease of
other LNAA not directly related to the enzyme defects. One characteristic shared by both the
elevated and decreased amino acids is that all are substrates for transport via the large
neutral amino acid transporter. In this study, the blood brain barrier was effectively bypassed
using microdialysis to determine the immediate effect of infused phenylalanine, tyrosine, 2 …
