Cytoskeleton of human mononuclear cells as a possible peripheral marker for phenylalanine neurotoxicity in PKU.
In this work we tested human mononuclear cells as a peripheral marker to study neurotoxicity of phenylalanine (Phe). Slices of cerebral cortex of rats or human mononuclear cells were incubated with different concentrations of Phe and/or Ala in the presence of 32P-orthophosphate, the cytoskeletal fraction was extracted, and the radioactivity incorporated into intermediate filament proteins was measured. Our results show that 2 mM Phe as well as 1 mM Ala are effective in increasing the 32P in vitro incorporation into IFs in both tissues. When cerebral cortex slices or mononuclear cells were incubated with different concentrations of Phe and/or Ala, the effects on the 32P in vitro incorporation into IF proteins was compatible with an antagonistic mechanism of action of the two amino acids on the enzymes of the phosphorylating system. In addition, these blood cells may be a possible peripheral marker to study neurotoxicity of Phe in patients with PKU.
Dick
Sabrina
S
Universidade Federal do Rio Grande do Sul, Instituto de Ciências Básicas da Saúde, Departamento de Bioquímica, Porto Alegre, RS Brazil.
Funchal
Cláudia
C
Pelaez
Priscila de Lima
Pde L
Loureiro
Samanta Oliveira
SO
Vivian
Lilian
L
Pessutto
Franciele Dall Bello
FD
Almeida
Lúcia Maria
LM
Wannmacher
Clóvis Milton Duval
CM
Pessoa-Pureur
Regina
R
D000818:Animals; D015415:Biomarkers; D003599:Cytoskeleton / Q000378:metabolism*; D004591:Electrophoresis, Polyacrylamide Gel; D006801:Humans; D066298:In Vitro Techniques; D009000:Monocytes / Q000378:metabolism*; D010649:Phenylalanine / Q000633:toxicity*; D010661:Phenylketonurias / Q000097:blood*; D051381:Rats; D017208:Rats, Wistar
D015415:Biomarkers; D010649:Phenylalanine