Abstract:Impaired proteostasis has been linked to various diseases, whereas little is known about the impact of peripherally misfolded proteins on the brain. We here studied the brain of mice with cardiomyocyte-restricted overexpression of a missense (R120G) mutant small heat shock protein, α B-crystallin (CryAB R120G ). At baseline, the CryAB R120G mice showed impaired cognitive and motor functions, aberrant protein aggregates, neuroinflammation, impaired bloodbrain barrier, and reduced proteasome activity in the brai… Show more
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