TDP‐43 Cryptic RNAs in Perry Syndrome: Differences across Brain Regions and TDP‐43 Proteinopathies

Abstract excerpt

BACKGROUND: Perry syndrome (PS) is a rare and fatal hereditary autosomal dominant neurodegenerative disorder caused by mutations in dynactin (DCTN1). PS brains accumulate inclusions positive for ubiquitin, transactive-response DNA-binding protein of 43 kDa (TDP-43), and to a lesser extent dynactin. OBJECTIVES: Little is known regarding the contributions of TDP-43, an RNA binding protein that represses cryptic...

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