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Article

Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids.

Advanced science (Weinheim, Baden-Wurttemberg, Germany) - 1 May 2024

Chen Anbin, Yangzom Tsering, Hong Yu, Lundberg Bjørn Christian, Sullivan Gareth John, Tzoulis Charalampos, Bindoff Laurence A, Liang Kristina Xiao

Abstract excerpt

In this research, a 3D brain organoid model is developed to study POLG-related encephalopathy, a mitochondrial disease stemming from POLG mutations. Induced pluripotent stem cells (iPSCs) derived from patients with these mutations is utilized to generate cortical organoids, which exhibited typical features of the diseases with POLG mutations, such as altered morphology, neuronal loss, and mitochondiral DNA...

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Brain
DNA, Mitochondrial
DNA Polymerase gamma
POLG gene
Induced Pluripotent Stem Cells

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Mitochondrial Diseases

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Open in PubMed Open full text

Identifiers

PMID: 38445970
DOI: 10.1002/advs.202307136
PMCID: PMC11095234

Genes

POLG

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 1 Sept 2026.

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Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids.PMID 38445970Read ↗

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