Related Experiment Video
Updated: Jan 7, 2026
07:47
Analysis of Brain Mitochondria Using Serial Block-Face Scanning Electron Microscopy
Published on: July 9, 2016
14.5K
An Emerging Role for OGDHL: From Mitochondrial Energy Metabolism to Neurodevelopmental Disorders
Xian Liu1, Guicheng Zhang1, Decai Yu2,3
1School of Life Science and Technology, The Key Laboratory of Developmental Genes and Human Disease, Southeast University, 2 Dongda Road, Nanjing 210031, China.
Biology
|December 30, 2025
Summary
Mutations in the oxoglutarate dehydrogenase-like (OGDHL) gene disrupt mitochondrial energy metabolism, causing neurodevelopmental disorders. This review details OGDHL
Area of Science:
- Biochemistry and Molecular Biology
- Neuroscience
- Genetics
Background:
- The oxoglutarate dehydrogenase-like (OGDHL) gene is crucial for mitochondrial energy metabolism in the brain.
- OGDHL mutations are associated with various neurodevelopmental disorders, including intellectual disability and epilepsy.
Purpose of the Study:
- To systematically review the discovery, structure, and function of OGDHL.
- To catalog pathogenic OGDHL mutations and their associated clinical phenotypes.
- To highlight OGDHL's role in neural development and disease.
Main Methods:
- Systematic literature review.
- Analysis of reported OGDHL mutations and clinical data.
- Synthesis of information on OGDHL structure and function.
Main Results:
- OGDHL is a key enzyme in the tricarboxylic acid cycle and brain energy metabolism.
- A comprehensive catalog of OGDHL mutations and their linked neurodevelopmental phenotypes is presented.
- OGDHL is implicated as a regulator of neural development and function.
Conclusions:
- OGDHL mutations disrupt mitochondrial function, leading to neurodevelopmental disorders.
- Understanding OGDHL is vital for comprehending metabolic regulation in neurodevelopment.
- This review provides a foundation for investigating OGDHL-related neurological diseases.
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