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Article

The NAD+ Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers' Disease.

International journal of biological sciences - 1 Jan 2024

Hong Yu, Zhang Zhuoyuan, Yangzom Tsering, Chen Anbin, Lundberg Bjørn Christian, Fang Evandro Fei, Siller Richard, Sullivan Gareth John, Zeman Jiri, Tzoulis Charalampos, Bindoff Laurence A, Liang Kristina Xiao

Abstract excerpt

Alpers' syndrome is an early-onset neurodegenerative disorder usually caused by biallelic pathogenic variants in the gene encoding the catalytic subunit of polymerase-gamma (POLG), which is essential for mitochondrial DNA (mtDNA) replication. The disease is progressive, incurable, and inevitably it leads to death from drug-resistant status epilepticus. The neurological features of Alpers' syndrome are intractable...

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Share to Diffuse Cerebral Sclerosis of Schilder
Share to DNA, Mitochondrial

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to DNA Polymerase gamma

Share to Induced Pluripotent Stem Cells

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Source

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Identifiers

PMID: 38385069
DOI: 10.7150/ijbs.91624
PMCID: PMC10878163

Genes

POLG

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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The NAD+ Precursor Nicotinamide Riboside Rescues Mitochondrial Defects and Neuronal Loss in iPSC derived Cortical Organoid of Alpers' Disease.PMID 38385069Read ↗

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