disease 2,530 words KG: ent-dise-7021bd98 2026-03-23
kind:diseasesection:diseasesstate:published
Contents

Cerebral Amyloid Angiopathy (CAA)

Disease Info
Aβ40 predominanceWhile Aβ42 is more aggregation-prone and found in parenchymal plaques, Aβ40 is the predominant isoform in CAA due to its higher affinity for cerebral blood vessel walls[@herzig2007]
APOE4 associationThe APOE ε4 allele strongly increases CAA risk and severity
Vascular localizationAmyloid deposits are found primarily in the:
Cytoplasmic vacuolizationEarly indicator of Aβ-induced injury
Nuclear pyknosisEvidence of apoptotic cell death
Complete cell lossAdvanced CAA shows near-complete VSMC replacement by amyloid
Media thinningVessel wall thickness increases while smooth muscle content decreases[@weller2015]
Contractile dysfunctionLoss of contractile phenotype, transitioning to synthetic state
Impaired autoregulationReduced ability to maintain constant cerebral blood flow
Dysregulated matrix maintenanceAltered production of extracellular matrix proteins
Endothelial decouplingLoss of endothelial-VSMC signaling coordination[@blair2021]
Direct pericyte bindingAβ peptides bind to pericyte surface receptors including CD36 and RAGE
DatabasesOMIMOrphanetClinicalTrialsPubMed

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