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000916472 0247_ $$2doi$$a10.1016/j.ijbiomac.2022.09.062
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000916472 1001_ $$0P:(DE-HGF)0$$aRondelli, Valeria$$b0$$eCorresponding author
000916472 245__ $$aDysmyelination and glycolipid interference caused by phenylalanine in phenylketonuria
000916472 260__ $$aNew York, NY [u.a.]$$bElsevier$$c2022
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000916472 520__ $$aPhenylketonuria (PKU) is a metabolic disorder connected to an excess of phenylalanine (Phe) in the blood and tissues, with neurological consequences. The disease's molecular bases seem to be related to the accumulation of Phe at the cell membrane surface. Radiological outcomes in the brain demonstrate decreased water diffusivity in white matter, involving axon dysmyelination of not yet understood origin. We used a biophysical approach and model membranes to extend our knowledge of Phe–membrane interaction by clarifying Phe's propensity to affect membrane structure and dynamics based on lipid composition, with emphasis on modulating cholesterol and glycolipid components to mimic raft domains and myelin sheath membranes. Phe showed affinity for the investigated membrane mimics, mainly affecting the Phe-facing membrane leaflet. The surfaces of our neuronal membrane raft mimics were strong anchoring sites for Phe, showing rigidifying effects. From a therapeutic perspective, we further investigated the role of doxycycline, known to disturb Phe packing, unveiling its action as a competitor in Phe interactions with the membrane, suggesting its potential for treatment in the early stages of PKU. Our results suggest how Phe accumulation in extracellular fluids can impede normal growth of myelin sheaths by interfering with membrane slipping and by remodulating free water and myelin-associated water contents.
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000916472 7001_ $$0P:(DE-Juel1)158075$$aKoutsioumpas, Alexandros$$b1
000916472 7001_ $$0P:(DE-HGF)0$$aDi Cola, Emanuela$$b2
000916472 7001_ $$0P:(DE-HGF)0$$aFragneto, Giovanna$$b3
000916472 7001_ $$0P:(DE-HGF)0$$aGrillo, I.$$b4
000916472 7001_ $$0P:(DE-HGF)0$$aDel Favero, Elena$$b5
000916472 7001_ $$0P:(DE-HGF)0$$aColombo, Laura$$b6
000916472 7001_ $$0P:(DE-HGF)0$$aCantù, Laura$$b7
000916472 7001_ $$0P:(DE-HGF)0$$aBrocca, Paola$$b8
000916472 7001_ $$0P:(DE-HGF)0$$aSalmona, Mario$$b9
000916472 773__ $$0PERI:(DE-600)1483284-7$$a10.1016/j.ijbiomac.2022.09.062$$gVol. 221, p. 784 - 795$$p784 - 795$$tInternational journal of biological macromolecules$$v221$$x0141-8130$$y2022
000916472 8564_ $$uhttps://juser.fz-juelich.de/record/916472/files/1-s2.0-S0141813022019900-main.pdf$$yOpenAccess
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