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Generation of Human Brain Organoids for Mitochondrial Disease Modeling
Published on: June 21, 2021
Developmental mechanisms and experimental models to understand forebrain malformative diseases.
A Pombero1, L Valdes, C Vieira
1Instituto de Neurociencias, UMH-CSIC, Campus de San Juan, Alicante, Spain.
Genes, Brain, and Behavior
|July 7, 2007
Summary
Genetic and epigenetic alterations during central nervous system development can cause brain disorders. This review details forebrain development, molecular mechanisms, and mouse models for human brain malformations.
Area of Science:
- Neuroscience
- Developmental Biology
- Genetics
Background:
- Central nervous system development involves distinct phases susceptible to genetic/epigenetic changes.
- Understanding vertebrate forebrain development has advanced, improving insights into brain malformations.
- Cortical malformations and neuronal migration disorders are key developmental brain issues.
Purpose of the Study:
- To review forebrain morphogenesis and regionalization stages.
- To emphasize molecular mechanisms disrupting telencephalic development and cortical function.
- To discuss mouse models crucial for studying human brain malformation diseases.
Main Methods:
- Literature review of forebrain development.
- Analysis of molecular and cellular mechanisms.
- Examination of established mouse models for brain malformations.
Main Results:
- Detailed description of forebrain development stages and regionalization.
- Identification of molecular pathways affecting telencephalic development.
- Highlighting the utility of mouse models in understanding human brain disorders.
Conclusions:
- Forebrain development is a complex process regulated by conserved molecular mechanisms.
- Mouse models are invaluable for dissecting the molecular basis of human brain malformations.
- Translating findings from mouse models to humans requires careful, system-specific analysis.
