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A new twist in CADASIL pathogenesis

ltbp_notchLatent TGF-β binding protein 1 (LTBP-1), an extracellular matrix protein and regulator of the TGF-β signaling pathway, was found to accumulate in CADASIL patient brain vessels and to co-localize with Notch3 deposits, a pathological hallmark of the disease.

Moreover, LTBP-1 was shown to directly interact with the Notch3 extracellular domain in vitro and to co-aggregate with CADASIL-mutant Notch3. Our findings highlight the importance of the extracellular matrix in small vessel disease and suggest a role of TGF-β signaling in disease pathogenesis. Read more...

Figure: Immunofluorescence co-staining of a CADASIL patient brain vessel

 

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