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Updated: Nov 17, 2025
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Ex Utero Electroporation and Organotypic Slice Culture of Mouse Hippocampal Tissue
Published on: March 4, 2015
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Usp11 controls cortical neurogenesis and neuronal migration through Sox11 stabilization
Shang-Yin Chiang1,2, Hsin-Chieh Wu1, Shu-Yu Lin1
1Institute of Biological Chemistry, Academia Sinica, Taipei 115, Taiwan.
Science Advances
|February 13, 2021
Summary
Protein stabilization by USP11 is crucial for brain development. USP11 deficiency impairs neuron production and migration, leading to cognitive and anxiety deficits in mice.
Area of Science:
- Neuroscience
- Developmental Biology
- Genetics
Background:
- The role of protein stabilization in cortical development is not well understood.
- A mutation in the USP11 gene is linked to a rare neurodevelopmental disorder, but its mechanism is unknown.
Purpose of the Study:
- To investigate the function of USP11 in cortical development.
- To elucidate the molecular mechanism underlying USP11's role in neurodevelopment.
Main Methods:
- Utilized a mouse model with Usp11 deficiency.
- Analyzed gene expression and protein levels in the embryonic cerebral cortex.
- Assessed neuronal production, migration, and cognitive behaviors.
Main Results:
- Usp11 deficiency in mice impairs layer 6 neuron production and neuronal migration.
- Sox11 was identified as a key substrate of Usp11, with Usp11 ablation reducing Sox11 protein accumulation.
- A disease-associated Usp11 mutant failed to stabilize Sox11 and rescue developmental defects.
Conclusions:
- USP11 plays a critical role in cortical development by stabilizing Sox11 protein.
- Protein stabilization is essential for regulating transcription and cell fate during brain development.
- Dysregulation of USP11 function contributes to neurodevelopmental disorders.
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