Patient-specific neural progenitor cells derived from induced pluripotent stem cells offer a promise of good models for mitochondrial disease.
Kristina Xiao LiangCecilie Katrin KristiansenGuro Helén VatneYu HongLaurence Albert BindoffPublished in: Cell and tissue research (2020)
Mitochondria are the primary generators of ATP in eukaryotic cells through the process of oxidative phosphorylation. Mitochondria are also involved in several other important cellular functions including regulation of intracellular Ca2+, cell signaling and apoptosis. Mitochondrial dysfunction causes disease and since it is not possible to perform repeated studies in humans, models are essential to enable us to investigate the mechanisms involved. Recently, the discovery of induced pluripotent stem cells (iPSCs), made by reprogramming adult somatic cells (Takahashi and Yamanaka 2006; Yamanaka and Blau 2010), has provided a unique opportunity for studying aspects of disease mechanisms in patient-specific cells and tissues. Reprogramming cells to neuronal lineage such as neural progenitor cells (NPCs) generated from the neural induction of reprogrammed iPSCs can thus provide a useful model for investigating neurological disease mechanisms including those caused by mitochondrial dysfunction. In addition, NPCs display a huge clinical potential in drug screening and therapeutics.
Keyphrases
- cell cycle arrest
- induced apoptosis
- induced pluripotent stem cells
- cell death
- oxidative stress
- signaling pathway
- gene expression
- small molecule
- single cell
- emergency department
- machine learning
- dna methylation
- young adults
- cell therapy
- bone marrow
- high throughput
- deep learning
- blood brain barrier
- genome wide
- protein kinase
- copy number