[HTML][HTML] Current approaches and future directions for the treatment of mTORopathies

V Karalis, HS Bateup - Developmental neuroscience, 2021 - karger.com
The mechanistic target of rapamycin (mTOR) is a kinase at the center of an evolutionarily
conserved signaling pathway that orchestrates cell growth and metabolism. mTOR responds …

Convergent and divergent mechanisms of epileptogenesis in mTORopathies

LH Nguyen, A Bordey - Frontiers in neuroanatomy, 2021 - frontiersin.org
Hyperactivation of the mechanistic target of rapamycin complex 1 (mTORC1) due to
mutations in genes along the PI3K-mTOR pathway and the GATOR1 complex causes a …

Human in vitro models of epilepsy using embryonic and induced pluripotent stem cells

MS Javaid, T Tan, N Dvir, A Anderson, T J. O'Brien… - Cells, 2022 - mdpi.com
The challenges in making animal models of complex human epilepsy phenotypes with
varied aetiology highlights the need to develop alternative disease models that can address …

STRADA‐mutant human cortical organoids model megalencephaly and exhibit delayed neuronal differentiation

LT Dang, S Vaid, G Lin, P Swaminathan… - Developmental …, 2021 - Wiley Online Library
Genetic diseases involving overactivation of the mechanistic target of rapamycin (mTOR)
pathway, so‐called “mTORopathies,” often manifest with malformations of cortical …

Mechanistic target of rapamycin signaling in human nervous system development and disease

M Girodengo, SK Ultanir, JM Bateman - Frontiers in Molecular …, 2022 - frontiersin.org
Mechanistic target of rapamycin (mTOR) is a highly conserved serine/threonine kinase that
regulates fundamental cellular processes including growth control, autophagy and …

Models of KPTN-related disorder implicate mTOR signalling in cognitive and overgrowth phenotypes

MO Levitin, LE Rawlins, G Sanchez-Andrade… - Brain, 2023 - academic.oup.com
KPTN-related disorder is an autosomal recessive disorder associated with germline variants
in KPTN (previously known as kaptin), a component of the mTOR regulatory complex …

[HTML][HTML] The molecular genetics of PI3K/PTEN/AKT/mTOR pathway in the malformations of cortical development

Q Ma, G Chen, Y Li, Z Guo, X Zhang - Genes & Diseases, 2024 - Elsevier
Malformations of cortical development (MCD) are a group of developmental disorders
characterized by abnormal cortical structures caused by genetic or harmful environmental …

Current review in basic science: animal models of focal cortical dysplasia and epilepsy

LH Nguyen, A Bordey - Epilepsy Currents, 2022 - journals.sagepub.com
Focal cortical dysplasia (FCD) is a malformation of cortical development that is a prevalent
cause of intractable epilepsy in children. Of the three FCD subtypes, understanding the …

In vitro human cell culture models in a bench‐to‐bedside approach to epilepsy

Š Danačíková, B Straka, J Daněk, V Kořínek… - Epilepsia …, 2024 - Wiley Online Library
Epilepsy is the most common chronic neurological disease, affecting nearly 1%–2% of the
world's population. Current pharmacological treatment and regimen adjustments are aimed …

Develo** novel experimental models of m-TORopathic epilepsy and related neuropathologies: translational insights from zebrafish

MS de Abreu, KA Demin, MM Kotova, F Mirzaei… - International Journal of …, 2023 - mdpi.com
The mammalian target of rapamycin (mTOR) is an important molecular regulator of cell
growth and proliferation. Brain mTOR activity plays a crucial role in synaptic plasticity, cell …