Explanipedia

U7 small nuclear RNA splice-switching therapeutics for STMN2 and UNC13A in Amyotrophic Lateral Sclerosis Open

Travis E. Solomon, Peter Harley, Christoph Schweingruber, Alessandro Marrero‐Gagliardi, Yujing Gao , et al. · 2025

TDP-43 nuclear depletion in amyotrophic lateral sclerosis (ALS) causes de-repression of cryptic exons (CEs) in multiple transcripts, including UNC13A and STMN2 , disrupting synaptic transmission and neurite outgrowth. We developed a therap…

Increased HCN1 activity in human excitatory neurons drives excessive network bursting in Dravet syndrome Open

Federica Riccio, Guilherme Neves, Michelle Gottlieb-Marra, Ariana Gatt, Christina E. Toomey , et al. · 2025

Dravet syndrome (DS) is a severe childhood epilepsy caused by mutations of the sodium channel NaV1.1. These mutations are thought to compromise the ability of inhibitory interneurons to regulate network activity, leading to seizure events.…

Unreliable homeostatic action potential broadening in cultured dissociated neurons Open

Andreas Ritzau‐Jost, Salil R. Rajayer, Jana Nerlich, Filip Maciąg, Alexandra John , et al. · 2025

Summary Homeostatic plasticity preserves neuronal activity against perturbations. Recently, somatic action potential broadening was proposed as a key homeostatic adaptation to chronic inactivity in neocortical neurons. Since action potenti…

Unreliable homeostatic action potential broadening in cultured dissociated neurons Open

Andreas Ritzau‐Jost, Salil R. Rajayer, Jana Nerlich, Filip Maciąg, Alexandra John , et al. · 2025

Summary Homeostatic plasticity preserves neuronal activity against perturbations. Recently, somatic action potential broadening was proposed as a key homeostatic adaptation to chronic inactivity in neocortical neurons. Since action potenti…

A workflow for semi‐automated volume correlative light microscopy and transmission electron tomography Open

K. Konishi, Guilherme Neves, Matthew R. G. Russell, Masafumi Mimura, Juan Burrone , et al. · 2025

Volume Correlative Light and Electron Microscopy (vCLEM) is a powerful method for assessing the ultrastructure of molecularly defined subcellular domains. A central challenge in vCLEM has been the efficient navigation of Regions of Interes…

Benchmark TEM data of mouse brain previously imaged by Nikon confocal microscopy Open

Konishi KK, Guilherme Neves, Matthew R. G. Russell, Juan Burrone, Roland A. Fleck · 2025

Unreliable homeostatic action potential broadening in cultured dissociated neurons Open

Andreas Ritzau‐Jost, Salil R. Rajayer, Jana Nerlich, Filip Maciąg, Alexandra John , et al. · 2025

Summary Homeostatic plasticity preserves neuronal activity against perturbations. Recently, somatic action potential broadening was proposed as a key homeostatic adaptation to chronic inactivity in neocortical neurons. Since action potenti…

Semi-automated navigation for efficient targeting of electron tomography to regions of interest in volume correlative light and electron microscopy Open

K. Konishi, Guilherme Neves, Matthew R. G. Russell, Masafumi Mimura, Juan Burrone , et al. · 2024

Electron microscopy is essential for the quantitative study of synaptic ultrastructure. At present, the correlation of functional and structural properties of the same synapse is extremely challenging. We introduce a novel integrated workf…

In vivo modulation of network activity drives the nanoscale reorganisation of axo-axonic synapses at the axon initial segment Open

Benjamin Compans, Vincenzo Mastrolia, Clara Lenherr, Juan Burrone · 2024

Chemical synapses control their strength through the nanoscale clustering of postsynaptic receptors into sub-synaptic domains (SSDs). Despite their importance in synapse function, the properties and plasticity of these domains are not well…

Loss of TDP-43 induces synaptic dysfunction that is rescued by UNC13A splice-switching ASOs Open

Matthew J. Keuss, Peter Harly, Eugeni Ryadnov, Rachel E. Jackson, Matteo Zanovello , et al. · 2024

TDP-43 loss of function induces multiple splicing changes, including a cryptic exon in the amyotrophic lateral sclerosis and fronto-temporal lobar degeneration risk gene UNC13A, leading to nonsense-mediated decay of UNC13A transcripts and …

Excitatory and inhibitory synapses show a tight subcellular correlation that weakens over development Open

Sally Horton, Vincenzo Mastrolia, Rachel E. Jackson, Sarah Kemlo, Pedro Machado , et al. · 2024

Neurons receive correlated levels of excitation and inhibition, a feature that is important for proper brain function. However, how this relationship between excitatory and inhibitory inputs is established during the dynamic period of circ…

Beyond a Transmission Cable—New Technologies to Reveal the Richness in Axonal Electrophysiology Open

José C. Mateus, Mónica Mendes Sousa, Juan Burrone, Paulo Aguiar · 2024

The axon is a neuronal structure capable of processing, encoding, and transmitting information. This assessment contrasts with a limiting, but deeply rooted, perspective where the axon functions solely as a transmission cable of somatodend…

Image Registration Based Navigation of Region of Interests in Volume Correlative Light and Electron Microscopy Open

K. Konishi, Guilherme Neves, Matthew R. G. Russell, Leanne Allison, Masafumi Mimura , et al. · 2024

Backscattered electron and x-ray imaging for array tomography provides rapid specimen characterisation and roi targeting Open

P. Machado, George Stonadge, Haithem Mansour, Alejandra Carbajal, Guilherme Neves , et al. · 2024

Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons Open

Peter Harley, Caoimhe Kerins, Ariana Gatt, Guilherme Neves, Federica Riccio , et al. · 2023

Dysregulated neuronal excitability is a hallmark of amyotrophic lateral sclerosis (ALS). We sought to investigate how functional changes to the axon initial segment (AIS), the site of action potential generation, could impact neuronal exci…

Cholinergic Stimulation Modulates the Functional Composition of CA3 Cell Types in the Hippocampus Open

Christopher Jon Puhl, Winnie Wefelmeyer, Juan Burrone · 2023

The functional heterogeneity of hippocampal CA3 pyramidal neurons has emerged as a key aspect of circuit function. Here, we explored the effects of long-term cholinergic activity on the functional heterogeneity of CA3 pyramidal neurons in …

Spatial subdomains in the Optic Tectum for the encoding of visual information Open

Thomas Shallcross, Giovanni Diana, Juan Burrone, Martin P. Meyer · 2023

1 Abstract Neurons across the visual system provide estimates of the visual features they encode. However, the reliability of those estimates can vary across the neuronal population. Here, we use information theory to provide a spatial map…

Multi-synaptic boutons are a feature of CA1 hippocampal connections in the stratum oriens Open

Mark Rigby, Federico W. Grillo, Benjamin Compans, Guilherme Neves, Júlia V. Gallinaro , et al. · 2023

Chandelier cells shine a light on the formation of GABAergic synapses Open

Benjamin Compans, Juan Burrone · 2023

Uncovering the wiring rules employed by neurons during development represents a formidable challenge with important repercussions for neurodevelopmental disorders. Chandelier cells (ChCs) are a singular GABAergic interneuron type, with a u…

Corrigendum: Correlative Live-Cell and Super-Resolution Imaging to Link Presynaptic Molecular Organisation With Function Open

Rachel E. Jackson, Benjamin Compans, Juan Burrone · 2022

[This corrects the article DOI: 10.3389/fnsyn.2022.830583.].

Multi-synaptic boutons are a feature of CA1 hippocampal connections that may underlie network synchrony Open

Mark Rigby, Federico W. Grillo, Benjamin Compans, Guilherme Neves, Júlia V. Gallinaro , et al. · 2022

Excitatory synapses are typically described as single synaptic boutons (SSBs), where one presynaptic bouton contacts a single postsynaptic spine. Using serial section block face scanning electron microscopy, we found that this textbook def…

Pathogenic TDP-43 Disrupts Axon Initial Segment Structure and Neuronal Excitability in a Human iPSC Model of ALS Open

Peter Harley, Guilherme Neves, Federica Riccio, Carolina Barcellos Machado, Aimee Cheesbrough , et al. · 2022

Dysregulated neuronal excitability is a hallmark of amyotrophic lateral sclerosis (ALS). We sought to investigate how functional changes to the axon initial segment (AIS), the site of action potential generation, could impact neuronal exci…

Cholinergic stimulation modulates the functional composition of CA3 cell types in the hippocampus Open

Christopher Jon Puhl, Winnie Wefelmeyer, Juan Burrone · 2022

The functional heterogeneity of hippocampal CA3 pyramidal neurons has emerged as a key aspect of circuit function. Here, we explored the effects of long-term cholinergic activity on the functional heterogeneity of CA3 pyramidal neurons in …

Correlative Live-Cell and Super-Resolution Imaging to Link Presynaptic Molecular Organisation With Function Open

Rachel E. Jackson, Benjamin Compans, Juan Burrone · 2022

Information transfer at synapses occurs when vesicles fuse with the plasma membrane to release neurotransmitters, which then bind to receptors at the postsynaptic membrane. The process of neurotransmitter release varies dramatically betwee…

Voltage-Gated Potassium Channels Ensure Action Potential Shape Fidelity in Distal Axons Open

Victoria Gonzalez Sabater, Mark Rigby, Juan Burrone · 2021

The initiation and propagation of the action potential (AP) along an axon allows neurons to convey information rapidly and across distant sites. Although AP properties have typically been characterized at the soma and proximal axon, knowle…

Drugs that inhibit TMEM16 proteins block SARS-CoV-2 spike-induced syncytia Open

Luca Braga, Hashim Ali, Ilaria Secco, Elena Chiavacci, Guilherme Neves , et al. · 2021

COVID-19 is a disease with unique characteristics that include lung thrombosis¹, frequent diarrhoea², abnormal activation of the inflammatory response³ and rapid deterioration of lung function consistent wi…

Voltage-gated potassium channels ensure action potential shape fidelity in distal axons Open

Victoria Gonzalez Sabater, Mark Rigby, Juan Burrone · 2020

The initiation and propagation of the action potential (AP) along an axon allows neurons to convey information rapidly and across distant sites. Although AP properties have typically been characterised at the soma and proximal axon, the pr…

Activity-Dependent Plasticity of Axo-axonic Synapses at the Axon Initial Segment Open

Alejandro Pan-Vazquez, Winnie Wefelmeyer, Victoria Gonzalez Sabater, Guilherme Neves, Juan Burrone · 2020

Transplantation of Chemogenetically Engineered Cortical Interneuron Progenitors into Early Postnatal Mouse Brains Open

Myrto Denaxa, Guilherme Neves, Juan Burrone, Vassilis Pachnis · 2019

Neuronal development is regulated by a complex combination of environmental and genetic factors. Assessing the relative contribution of each component is a complicated task, which is particularly difficult in regards to the development of …

In Vitro Modeling of Nerve–Muscle Connectivity in a Compartmentalized Tissue Culture Device Open

Carolina Barcellos Machado, Perrine Pluchon, Peter Harley, Mark Rigby, Victoria Gonzalez Sabater , et al. · 2019

Motor neurons project axons from the hindbrain and spinal cord to muscle, where they induce myofibre contractions through neurotransmitter release at neuromuscular junctions. Studies of neuromuscular junction formation and homeostasis have…

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