Altered brain protein expression profiles are associated with molecular neurological dysfunction in the PKU mouse model.
Bottom Line: Our results indicate that differential expression of these proteins may be associated with the processes underlying PKU brain dysfunction, namely, decreased synaptic plasticity and impaired neurotransmission.We think that phenylketonuria (PKU) brain dysfunction also depends on reduced Syn2 and Dpysl2 levels, increased Glu2/3 and NR1 levels, and decreased Pkm, Ckb, Pgam1 and Eno1 levels.These findings finally confirm that alteration in synaptic function, in transmission and in energy metabolism underlie brain damage provoked by hyperphenylalaninemias.
Affiliation: IRCCS SDN-Foundation, Naples, Italy.Show MeSH
Related in: MedlinePlus
Mentions: We also evaluated the protein levels of the Glu2/3 α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor subunit and the NMDA receptor subunit NR1 in the control and PKU mouse brain extracts. Western blot and densitometric analyses showed that the expressions of the Glu2/3 and NR1 receptor subunits were significantly up-regulated in PKU mouse brain (Fig. 6a and b).