[HTML][HTML] Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis

J Nijssen, LH Comley, E Hedlund - Acta neuropathologica, 2017 - Springer
In the fatal disease—amyotrophic lateral sclerosis (ALS)—upper (corticospinal) motor
neurons (MNs) and lower somatic MNs, which innervate voluntary muscles, degenerate. …

[PDF][PDF] Axon-seq decodes the motor axon transcriptome and its modulation in response to ALS

J Nijssen, J Aguila, R Hoogstraaten, N Kee, E Hedlund - Stem cell reports, 2018 - cell.com
Spinal motor axons traverse large distances to innervate target muscles, thus requiring local
control of cellular events for proper functioning. To interrogate axon-specific processes we …

Fatal demyelinating disease is induced by monocyte-derived macrophages in the absence of TGF-β signaling

…, E Ewing, L Kular, J Han, K Zhu, J Nijssen… - Nature …, 2018 - nature.com
The cytokine transforming growth factor-β (TGF-β) regulates the development and homeostasis
of several tissue-resident macrophage populations, including microglia. TGF-β is not …

LCM-seq reveals unique transcriptional adaptation mechanisms of resistant neurons and identifies protective pathways in spinal muscular atrophy

S Nichterwitz, J Nijssen, H Storvall… - Genome …, 2020 - genome.cshlp.org
Somatic motor neurons are selectively vulnerable in spinal muscular atrophy (SMA), which
is caused by a deficiency of the ubiquitously expressed survival of motor neuron protein. …

Cross‐disease comparison of amyotrophic lateral sclerosis and spinal muscular atrophy reveals conservation of selective vulnerability but differential neuromuscular …

LH Comley, J Nijssen, J Frost‐Nylen… - Journal of …, 2016 - Wiley Online Library
Neuromuscular junctions are primary pathological targets in the lethal motor neuron
diseases spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS). Synaptic …

Disrupted function of lactate transporter MCT1, but not MCT4, in Schwann cells affects the maintenance of motor end‐plate innervation

…, G Pollmeier, K Sandor, C Morado Urbina, J Nijssen… - Glia, 2021 - Wiley Online Library
Recent studies in neuron‐glial metabolic coupling have shown that, in the CNS, astrocytes
and oligodendrocytes support neurons with energy‐rich lactate/pyruvate via …

[PDF][PDF] Muscle-secreted neurturin couples myofiber oxidative metabolism and slow motor neuron identity

…, I Cervenka, N Khan, M Stec, M Oliveira, J Nijssen… - Cell Metabolism, 2021 - cell.com
Endurance exercise promotes skeletal muscle vascularization, oxidative metabolism, fiber-type
switching, and neuromuscular junction integrity. Importantly, the metabolic and …

[PDF][PDF] Modeling motor neuron resilience in ALS using stem cells

I Allodi, J Nijssen, JA Benitez, C Schweingruber… - Stem cell reports, 2019 - cell.com
Oculomotor neurons, which regulate eye movement, are resilient to degeneration in the
lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly …

Intra-condensate demixing of TDP-43 inside stress granules generates pathological aggregates

X Yan, D Kuster, P Mohanty, J Nijssen… - bioRxiv, 2024 - biorxiv.org
Cytosolic aggregation of the nuclear protein TDP-43 is associated with many
neurodegenerative diseases, but the triggers for TDP-43 aggregation are still debated. Here, we …

Single-Cell mRNA-Seq of In Vitro-Derived Human Neurons Using Smart-Seq2

C Schweingruber, J Nijssen, JA Benitez… - Transcription Factor …, 2022 - Springer
Single-cell mRNA sequencing can dissect heterogeneous cell populations as it can identify
cell types and cellular states based on their unique transcriptional signatures. We use …