Hereditary Optic Neuropathies: Induced Pluripotent Stem Cell-Based 2D/3D Approaches.
Marta García-LópezJoaquín ArenasMaría Esther GallardoPublished in: Genes (2021)
Inherited optic neuropathies share visual impairment due to the degeneration of retinal ganglion cells (RGCs) as the hallmark of the disease. This group of genetic disorders are caused by mutations in nuclear genes or in the mitochondrial DNA (mtDNA). An impaired mitochondrial function is the underlying mechanism of these diseases. Currently, optic neuropathies lack an effective treatment, and the implementation of induced pluripotent stem cell (iPSC) technology would entail a huge step forward. The generation of iPSC-derived RGCs would allow faithfully modeling these disorders, and these RGCs would represent an appealing platform for drug screening as well, paving the way for a proper therapy. Here, we review the ongoing two-dimensional (2D) and three-dimensional (3D) approaches based on iPSCs and their applications, taking into account the more innovative technologies, which include tissue engineering or microfluidics.
Keyphrases
- mitochondrial dna
- stem cells
- copy number
- tissue engineering
- optical coherence tomography
- high glucose
- genome wide
- diabetic rats
- induced apoptosis
- drug induced
- optic nerve
- healthcare
- high throughput
- emergency department
- gene expression
- endothelial cells
- cell therapy
- dna methylation
- mesenchymal stem cells
- endoplasmic reticulum stress
- cell death
- signaling pathway
- smoking cessation
- combination therapy
- bioinformatics analysis
- electronic health record