Age-dependent vascular and neurological characteristics of CADASIL are recapitulated in Notch3 mutant zebrafish, implicating a role for type IV collagen in disease progression.

Abstract excerpt

CADASIL is a hereditary cerebral small vessel disease caused by NOTCH3 mutations, leading to age-dependent vascular and neurological impairments. Despite its clinical impact, the underlying pathogenic mechanisms remain poorly understood, partly because existing murine models often fail to fully replicate the broad pathological spectrum observed in humans. Here, we established and characterized a novel zebrafish...

Topics

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• Animals, Genetically Modified
• Brain
• CADASIL
• Collagen Type IV