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@ARTICLE{Rondelli:916472,
      author       = {Rondelli, Valeria and Koutsioumpas, Alexandros and Di Cola,
                      Emanuela and Fragneto, Giovanna and Grillo, I. and Del
                      Favero, Elena and Colombo, Laura and Cantù, Laura and
                      Brocca, Paola and Salmona, Mario},
      title        = {{D}ysmyelination and glycolipid interference caused by
                      phenylalanine in phenylketonuria},
      journal      = {International journal of biological macromolecules},
      volume       = {221},
      issn         = {0141-8130},
      address      = {New York, NY [u.a.]},
      publisher    = {Elsevier},
      reportid     = {FZJ-2022-06264},
      pages        = {784 - 795},
      year         = {2022},
      abstract     = {Phenylketonuria (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.},
      cin          = {JCNS-FRM-II / MLZ / JCNS-1 / JCNS-4},
      ddc          = {570},
      cid          = {I:(DE-Juel1)JCNS-FRM-II-20110218 / I:(DE-588b)4597118-3 /
                      I:(DE-Juel1)JCNS-1-20110106 / I:(DE-Juel1)JCNS-4-20201012},
      pnm          = {6G4 - Jülich Centre for Neutron Research (JCNS) (FZJ)
                      (POF4-6G4) / 632 - Materials – Quantum, Complex and
                      Functional Materials (POF4-632)},
      pid          = {G:(DE-HGF)POF4-6G4 / G:(DE-HGF)POF4-632},
      experiment   = {EXP:(DE-MLZ)MARIA-20140101},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {36099998},
      UT           = {WOS:000860501900004},
      doi          = {10.1016/j.ijbiomac.2022.09.062},
      url          = {https://juser.fz-juelich.de/record/916472},
}