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Related Experiment Video

Updated: May 15, 2025

Measuring Enzymatic Activity of Neurodevelopmental Disorder-Associated Deubiquitylating Enzymes via an In Vitro Ubiquitin Chain Cleavage Assay

Measuring Enzymatic Activity of Neurodevelopmental Disorder-Associated Deubiquitylating Enzymes via an In Vitro Ubiquitin Chain Cleavage Assay

Published on: September 27, 2024

Cullin-RING Ubiquitin Ligases in Neurodevelopment and Neurodevelopmental Disorders.

Honoka Ashitomi¹, Tadashi Nakagawa^1,2, Makiko Nakagawa^3,4

¹Department of Clinical Pharmacology, Faculty of Pharmaceutical Sciences, Sanyo-Onoda City University, Sanyo-Onoda 756-0084, Japan.

|April 29, 2025

Summary

Ubiquitin ligases are crucial for brain development and function. Dysregulation of these enzymes, particularly Cullin-RING ubiquitin ligases, is linked to neurodevelopmental disorders, offering potential therapeutic targets.

Keywords:

Cullin-RING ubiquitin ligase neurodevelopment neurodevelopmental disorders

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Last Updated: May 15, 2025

Measuring Enzymatic Activity of Neurodevelopmental Disorder-Associated Deubiquitylating Enzymes via an In Vitro Ubiquitin Chain Cleavage Assay

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Published on: September 27, 2024

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Published on: May 14, 2021

A Scalable, Cell-Based Method for the Functional Assessment of Ube3a Variants

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Published on: October 10, 2022

Area of Science:

Biochemistry
Molecular Biology
Neuroscience

Background:

Ubiquitination is a vital post-translational modification regulating protein function and cellular homeostasis.
E3 ubiquitin ligases are key enzymes controlling substrate specificity in the ubiquitination cascade.
These ligases play critical roles in neurodevelopmental processes.

Purpose of the Study:

To review the molecular mechanisms of neurodevelopment regulated by Cullin-RING ubiquitin ligases.
To explore the involvement of these ligases in the pathophysiology of neurodevelopmental disorders.
To highlight the therapeutic potential of understanding these pathways.

Main Methods:

Literature review synthesizing current knowledge.
Focus on Cullin-RING ubiquitin ligases, the largest subclass.
Analysis of their role in neurodevelopment and associated disorders.

Main Results:

Cullin-RING ubiquitin ligases regulate essential neurodevelopmental processes like neuronal polarization and synaptogenesis.
Mutations and dysregulation of these ligases are implicated in autism spectrum disorder, intellectual disability, and ADHD.
Understanding these mechanisms provides insights into disease pathology.

Conclusions:

Cullin-RING ubiquitin ligases are critical regulators of neurodevelopment.
Their dysregulation contributes to neurodevelopmental disorders.
Targeting these pathways may lead to novel therapeutic interventions.