High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria

Schumacher U, Lukacs Z, Kaltschmidt C, Freudlsperger C, Schulz D, Kompisch K, Mueller R, Rudolph T, Santer R, Lorke DE, Ullrich K (2008)
NEUROBIOLOGY OF DISEASE 32(3): 385-390.

Zeitschriftenaufsatz | Veröffentlicht | Englisch
 
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DOI
Autor*in
Schumacher, Udo; Lukacs, Zoltan; Kaltschmidt, ChristianUniBi; Freudlsperger, Christian; Schulz, Dorothea; Kompisch, Kai; Mueller, Reinhard; Rudolph, Tanja; Santer, Rene; Lorke, Dietrich E.; Ullrich, Kurt
Einrichtung
Centrum für Biotechnologie > Arbeitsgruppe C. Kaltschmidt
Fakultät für Biologie > Zellbiologie der Tiere
Centrum für Biotechnologie > Institut für Genomforschung und Systembiologie
Abstract / Bemerkung
If left untreated, the common inherited metabolic disorder phenylketonuria (PKU) presents with mental retardation and reduced brain weight. The underlying molecular reasons for these deficits are unknown so far. Using human neuroblastoma cells as a model for normal human neuroblasts elevated phenylalanine, concentrations suppressed proliferation of these cells in culture. Furthermore, microarray and functional assays of these cells revealed that both phenylalanine and the known PPAR gamma agonist rosiglitazone regulated the same set of genes causing subsequently similar changes in the functional assays. The lowered brain weight of PKU patients may thus be the result of reduced neuroblast proliferation caused by phenylalanine-induced stimulation of PPAR gamma receptors. The observation that high concentrations of small substrates can activate receptors may serve as a new paradigm for other metabolic diseases and provides a new approach for the treatment of these disorders by application of specific receptor antagonists. (C) 2008 Elsevier Inc. All rights reserved.
Stichworte
Peroxisome proliferator-activated receptor gamma; Pathophysiology; Neuroblastoma cells; Phenylketonuria
Erscheinungsjahr
2008
Zeitschriftentitel
NEUROBIOLOGY OF DISEASE
Band
32
Ausgabe
3
Seite(n)
385-390
ISSN
0969-9961
Page URI
https://pub.uni-bielefeld.de/record/1636528

Zitieren

Schumacher U, Lukacs Z, Kaltschmidt C, et al. High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria. NEUROBIOLOGY OF DISEASE. 2008;32(3):385-390.
Schumacher, U., Lukacs, Z., Kaltschmidt, C., Freudlsperger, C., Schulz, D., Kompisch, K., Mueller, R., et al. (2008). High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria. NEUROBIOLOGY OF DISEASE, 32(3), 385-390. https://doi.org/10.1016/j.nbd.2008.07.020
Schumacher, Udo, Lukacs, Zoltan, Kaltschmidt, Christian, Freudlsperger, Christian, Schulz, Dorothea, Kompisch, Kai, Mueller, Reinhard, et al. 2008. “High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria”. NEUROBIOLOGY OF DISEASE 32 (3): 385-390.
Schumacher, U., Lukacs, Z., Kaltschmidt, C., Freudlsperger, C., Schulz, D., Kompisch, K., Mueller, R., Rudolph, T., Santer, R., Lorke, D. E., et al. (2008). High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria. NEUROBIOLOGY OF DISEASE 32, 385-390.
Schumacher, U., et al., 2008. High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria. NEUROBIOLOGY OF DISEASE, 32(3), p 385-390.
U. Schumacher, et al., “High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria”, NEUROBIOLOGY OF DISEASE, vol. 32, 2008, pp. 385-390.
Schumacher, U., Lukacs, Z., Kaltschmidt, C., Freudlsperger, C., Schulz, D., Kompisch, K., Mueller, R., Rudolph, T., Santer, R., Lorke, D.E., Ullrich, K.: High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria. NEUROBIOLOGY OF DISEASE. 32, 385-390 (2008).
Schumacher, Udo, Lukacs, Zoltan, Kaltschmidt, Christian, Freudlsperger, Christian, Schulz, Dorothea, Kompisch, Kai, Mueller, Reinhard, Rudolph, Tanja, Santer, Rene, Lorke, Dietrich E., and Ullrich, Kurt. “High concentrations of phenylalanine stimulate peroxisome proliferator-activated receptor gamma: Implications for the pathophysiology of phenylketonuria”. NEUROBIOLOGY OF DISEASE 32.3 (2008): 385-390.

5 Zitationen in Europe PMC

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