C9orf72-mediated ALS and FTD: multiple pathways to disease

R Balendra, AM Isaacs - Nature Reviews Neurology, 2018 - nature.com
The discovery that repeat expansions in the C9orf72 gene are a frequent cause of
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) has revolutionized …

Converging pathways in neurodegeneration, from genetics to mechanisms

L Gan, MR Cookson, L Petrucelli, AR La Spada - Nature neuroscience, 2018 - nature.com
Neurodegenerative diseases cause progressive loss of cognitive and/or motor function and
pose major challenges for societies with rapidly aging populations. Human genetics studies …

Disruption of RNA metabolism in neurological diseases and emerging therapeutic interventions

JK Nussbacher, R Tabet, GW Yeo, C Lagier-Tourenne - Neuron, 2019 - cell.com
RNA binding proteins are critical to the maintenance of the transcriptome via controlled
regulation of RNA processing and transport. Alterations of these proteins impact multiple …

Post-transcriptional regulation of gene expression and human disease

AH Corbett - Current opinion in cell biology, 2018 - Elsevier
A large number of mutations in genes that encode RNA binding proteins cause human
disease. Many of these RNA binding proteins mediate key steps in post-transcriptional …

Expansion of GGC repeat in GIPC1 is associated with oculopharyngodistal myopathy

J Deng, J Yu, P Li, X Luan, L Cao, J Zhao, M Yu… - The American Journal of …, 2020 - cell.com
Oculopharyngodistal myopathy (OPDM) is an adult-onset inherited neuromuscular disorder
characterized by progressive ptosis, external ophthalmoplegia, and weakness of the …

Gene therapy in amyotrophic lateral sclerosis

T Fang, G Je, P Pacut, K Keyhanian, J Gao, M Ghasemi - Cells, 2022 - mdpi.com
Since the discovery of Cu/Zn superoxide dismutase (SOD1) gene mutation, in 1993, as the
first genetic abnormality in amyotrophic lateral sclerosis (ALS), over 50 genes have been …

[HTML][HTML] In vivo genome editing in animals using AAV-CRISPR system: applications to translational research of human disease

CH Lau, Y Suh - F1000Research, 2017 - ncbi.nlm.nih.gov
Adeno-associated virus (AAV) has shown promising therapeutic efficacy with a good safety
profile in a wide range of animal models and human clinical trials. With the advent of …

Dead Cas systems: types, principles, and applications

S Brezgin, A Kostyusheva, D Kostyushev… - International journal of …, 2019 - mdpi.com
The gene editing tool CRISPR-Cas has become the foundation for developing numerous
molecular systems used in research and, increasingly, in medical practice. In particular, Cas …

[HTML][HTML] TCF4-mediated Fuchs endothelial corneal dystrophy: Insights into a common trinucleotide repeat-associated disease

MP Fautsch, ED Wieben, KH Baratz… - Progress in retinal and …, 2021 - Elsevier
Fuchs endothelial corneal dystrophy (FECD) is a common cause for heritable visual loss in
the elderly. Since the first description of an association between FECD and common …

Evolution of therapies for the corneal endothelium: past, present and future approaches

HS Ong, M Ang, J Mehta - British Journal of Ophthalmology, 2021 - bjo.bmj.com
Corneal endothelial diseases are leading indications for corneal transplantations. With
significant advancement in medical science and surgical techniques, corneal transplant …