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Reducing Timp3 or Vitronectin Ameliorates Disease Manifestations in CADASIL Mice

OBJECTIVE: CADASIL is a genetic paradigm of cerebral small vessel disease caused by NOTCH3 mutations that stereotypically lead to the extracellular deposition of NOTCH3 ectodomain (Notch3(ECD)) on the vessels. TIMP3 and vitronectin are 2 extracellular matrix proteins that abnormally accumulate in No...

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Bibliographische Detailangaben
Veröffentlicht in:Ann Neurol
Hauptverfasser: Capone, Carmen, Cognat, Emmanuel, Ghezali, Lamia, Baron-Menguy, Céline, Aubin, Déborah, Mesnard, Laurent, Stöhr, Heidi, Domenga-Denier, Valérie, Nelson, Mark T., Joutel, Anne
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2016
Schlagworte:
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5359978/
https://ncbi.nlm.nih.gov/pubmed/26648042
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/ana.24573
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