English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Plus monthly

Global: Zendy Tools annual

Global: Zendy Tools monthly

Global: Zendy Free Plan

Significance Because of the toxicity gain of expanded polyglutamine (polyQ) repeats, many studies have used RNAi and other approaches to inactivate the mutantHTT gene in Huntington’s disease. However, Htt is essential for early embryonic development, and normal function of Htt in adult animals remains unknown. Using conditional Htt knockout mice, we found that loss of Htt causes the lethal phenotype only in postnatal mice but not in adult mice, and this early death is due to acute pancreatitis. Htt interacts with serine protease inhibitor Kazal-type 3 (Spink3) to inhibit trypsin activity in pancreatic cells and thus prevents acinar cell degeneration and pancreatitis. This age- and cell type-dependent function indicates the safety in knocking down neuronal HTT in adult brains for treating Huntington’s disease.

Ablation of huntingtin in adult neurons is nondeleterious but its depletion in young mice causes acute pancreatitis