Voltage-gated calcium channels in nonexcitable tissues

GS Pitt, M Matsui, C Cao - Annual review of physiology, 2021 - annualreviews.org
The identification of a gain-of-function mutation in CACNA1C as the cause of Timothy
syndrome, a rare disorder characterized by cardiac arrhythmias and syndactyly, highlighted …

Calcium and activity-dependent signaling in the developing cerebral cortex

A Arjun McKinney, R Petrova… - Development, 2022 - journals.biologists.com
Calcium influx can be stimulated by various intra-and extracellular signals to set coordinated
gene expression programs into motion. As such, the precise regulation of intracellular …

[HTML][HTML] Dissecting the molecular basis of human interneuron migration in forebrain assembloids from Timothy syndrome

F Birey, MY Li, A Gordon, MV Thete, AM Valencia… - Cell stem cell, 2022 - cell.com
Defects in interneuron migration can disrupt the assembly of cortical circuits and lead to
neuropsychiatric disease. Using forebrain assembloids derived by integration of cortical and …

Disrupted Cacna1c gene expression perturbs spontaneous Ca2+ activity causing abnormal brain development and increased anxiety

E Smedler, L Louhivuori… - Proceedings of the …, 2022 - National Acad Sciences
The L-type voltage-gated Ca2+ channel gene CACNA1C is a risk gene for various
psychiatric conditions, including schizophrenia and bipolar disorder. However, the cellular …

Modeling human neurodevelopmental diseases with brain organoids

X Lu, J Yang, Y Xiang - Cell Regeneration, 2022 - Springer
Studying the etiology of human neurodevelopmental diseases has long been a challenging
task due to the brain's complexity and its limited accessibility. Human pluripotent stem cells …

Update on the molecular genetics of Timothy syndrome

R Bauer, KW Timothy, A Golden - Frontiers in pediatrics, 2021 - frontiersin.org
Timothy Syndrome (TS)(OMIM# 601005) is a rare autosomal dominant syndrome caused by
variants in CACNA1C, which encodes the α1C subunit of the voltage-gated calcium channel …

Making Ramón y Cajal proud: Development of cell identity and diversity in the cerebral cortex

DJ Di Bella, N Domínguez-Iturza, JR Brown, P Arlotta - Neuron, 2024 - cell.com
Since the beautiful images of Santiago Ramón y Cajal provided a first glimpse into the
immense diversity and complexity of cell types found in the cerebral cortex, neuroscience …

Voltage-Gated Ca2+-Channel α1-Subunit de novo Missense Mutations: Gain or Loss of Function – Implications for Potential Therapies

J Striessnig - Frontiers in synaptic neuroscience, 2021 - frontiersin.org
This review summarizes our current knowledge of human disease-relevant genetic variants
within the family of voltage gated Ca2+ channels. Ca2+ channelopathies cover a wide …

Antisense oligonucleotide therapeutic approach for Timothy syndrome

X Chen, F Birey, MY Li, O Revah, R Levy, MV Thete… - Nature, 2024 - nature.com
Timothy syndrome (TS) is a severe, multisystem disorder characterized by autism, epilepsy,
long-QT syndrome and other neuropsychiatric conditions. TS type 1 (TS1) is caused by a …

Patient-derived iPSC modeling of rare neurodevelopmental disorders: Molecular pathophysiology and prospective therapies

KR Sabitha, AK Shetty, D Upadhya - Neuroscience & Biobehavioral …, 2021 - Elsevier
The pathological alterations that manifest during the early embryonic development due to
inherited and acquired factors trigger various neurodevelopmental disorders (NDDs) …