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@ARTICLE{DosSantosMorais:856718,
author = {Dos Santos Morais, Raphael and Delalande, Olivier and
Pérez, Javier and Mias-Lucquin, Dominique and Lagarrigue,
Mélanie and Martel, Anne and Molza, Anne-Elisabeth and
Chéron, Angélique and Raguénès-Nicol, Céline and
Chenuel, Thomas and Bondon, Arnaud and Appavou, Marie-Sousai
and Le Rumeur, Elisabeth and Combet, Sophie and Hubert,
Jean-François},
title = {{H}uman {D}ystrophin {S}tructural {C}hanges upon {B}inding
to {A}nionic {M}embrane {L}ipids},
journal = {Biophysical journal},
volume = {115},
number = {7},
issn = {0006-3495},
address = {Bethesda, Md.},
publisher = {Soc.},
reportid = {FZJ-2018-06069},
pages = {1231 - 1239},
year = {2018},
abstract = {Scaffolding proteins play important roles in supporting the
plasma membrane (sarcolemma) of muscle cells.Among them,
dystrophin strengthens the sarcolemma through protein-lipid
interactions, and its absence due to gene mutationsleads to
the severe Duchenne muscular dystrophy. Most of the
dystrophin protein consists of a central domain made of 24
spec-trin-like coiled-coil repeats (R). Using small angle
neutron scattering (SANS) and the contrast variation
technique, we specificallyprobed the structure of the three
first consecutive repeats 1–3 (R1–3), a part of
dystrophin known to physiologically interact withmembrane
lipids. R1–3 free in solution was compared to its
structure adopted in the presence of phospholipid-based
bicelles.SANS data for the protein/lipid complexes were
obtained with contrast-matched bicelles under various
phospholipid composi-tions to probe the role of
electrostatic interactions. When bound to anionic bicelles,
large modifications of the protein three-dimensional
structure were detected, as revealed by a significant
increase of the protein gyration radius from 42 51 to60 54
A˚. R1–3/anionic bicelle complexes were further analyzed
by coarse-grained molecular dynamics simulations. Fromthese
studies, we report an all-atom model of R1–3 that
highlights the opening of the R1 coiled-coil repeat when
bound tothe membrane lipids. This model is totally in
agreement with SANS and click chemistry/mass spectrometry
data. We concludethat the sarcolemma membrane anchoring that
occurs during the contraction/elongation process of muscles
could be ensuredby this coiled-coil opening. Therefore,
understanding these structural changes may help in the
design of rationalized shorteneddystrophins for gene
therapy. Finally, our strategy opens up new possibilities
for structure determination of peripheral and inte-gral
membrane proteins not compatible with different
high-resolution structural methods.},
cin = {JCNS-FRM-II / Neutronenstreuung ; JCNS-1},
ddc = {570},
cid = {I:(DE-Juel1)JCNS-FRM-II-20110218 /
I:(DE-Juel1)JCNS-1-20110106},
pnm = {6G4 - Jülich Centre for Neutron Research (JCNS) (POF3-623)
/ 6G15 - FRM II / MLZ (POF3-6G15)},
pid = {G:(DE-HGF)POF3-6G4 / G:(DE-HGF)POF3-6G15},
experiment = {EXP:(DE-MLZ)KWS1-20140101 / EXP:(DE-MLZ)KWS2-20140101},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:30197181},
UT = {WOS:000446056300010},
doi = {10.1016/j.bpj.2018.07.039},
url = {https://juser.fz-juelich.de/record/856718},
}