[HTML][HTML] Delineating mechanisms underlying parvalbumin neuron impairment in different neurological and neurodegenerative disorders: the emerging role of …

EA Olkhova, LA Smith, BH Dennis… - Biochemical Society …, 2024 - portlandpress.com
Single-molecule imaging techniques have revealed the dynamic nature of ion channels and
shown that channel activity is sometimes dependent on their mobility and mechanical forces …

Role of Kir4. 1 Channels in Aminoglycoside‐Induced Ototoxicity of Hair Cells

JS Choi, YJ Ahn, SH Lee, DJ Park… - BioMed Research …, 2023 - Wiley Online Library
The Kir4. 1 channel, an inwardly rectifying potassium ion (K+) channel, is located in the hair
cells of the organ of Corti as well as the intermediate cells of the stria vascularis. The Kir4. 1 …

Hallmark molecular and pathological features of POLG disease are recapitulated in cerebral organoids

A Chen, T Yangzom, Y Hong, BC Lundberg… - Advanced …, 2024 - Wiley Online Library
In this research, a 3D brain organoid model is developed to study POLG‐related
encephalopathy, a mitochondrial disease stemming from POLG mutations. Induced …

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

Y Hong, Z Zhang, T Yangzom, A Chen… - … Journal of Biological …, 2024 - ncbi.nlm.nih.gov
Alpers' syndrome is an early-onset neurodegenerative disorder usually caused by biallelic
pathogenic variants in the gene encoding the catalytic subunit of polymerase-gamma …

[HTML][HTML] Resistance Exercise Training Rescues Mitochondrial Dysfunction in Skeletal Muscle of Patients with Myotonic Dystrophy Type 1

V Di Leo, C Lawless, MP Roussel… - Journal of …, 2023 - content.iospress.com
Background: Myotonic dystrophy type 1 (DM1) is a dominant autosomal neuromuscular
disorder caused by the inheritance of a CTG triplet repeat expansion in the Dystrophia …

[PDF][PDF] Research Article Role of Kir4. 1 Channels in Aminoglycoside-Induced Ototoxicity of Hair Cells

JS Choi, YJ Ahn, SH Lee, DJ Park, JE Park, SM Ha… - 2023 - researchgate.net
The Kir4. 1 channel, an inwardly rectifying potassium ion (K+) channel, is located in the hair
cells of the organ of Corti as well as the intermediate cells of the stria vascularis. The Kir4. 1 …