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ALTERED GRANULE DYNAMICS OF CDKL5 DEFICIENCY DURING DEVELOPMENT
Zhongyu Zhengand 4 co-authors
The Chinese University of Hong Kong
FENS Forum 2026 (2026)
Barcelona, Spain
Board PS04-08PM-552
Presentation
Date TBA
Board: PS04-08PM-552
Event Information
Poster Board
PS04-08PM-552
Poster
View posterAbstract
CDKL5 deficiency disorder (CDD) is an X-linked neurodevelopmental disorder causing by loss of function of a serine/threonine kinase, Cyclin-Dependent-Kinase-Like 5 (CDKL5). CDD patients suffer from severe neurodevelopment defects, and the underlying pathological mechanism is not clear. In previous studies we identified nELAVL as novel substrates of CDKL5. Lack of phosphorylation by CDKL5 leads to altered nELAVL phase separation in rodent models. We used CDD patient-derived neuron model and established an isogenic control by Adenine based editing (ABE). In CDD neurons, we found granule formation increased not only in nELAVL granules but also in P-bodies and stress granules, which suggested altered granule interaction in CDD neurons. We also found altered RNA processing caused by loss of CDKL5 phosphorylation on nELAVL. Our work suggest a novel mechanism that CDKL5 regulate RNA processing by altering granule dynamics through phosphorylation.
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