Selective vulnerability of spinal and cortical motor neuron subpopulations in delta7 SMA mice. [PDF]
PLoS ONE, 2013 Loss of the survival motor neuron gene (SMN1) is responsible for spinal muscular atrophy (SMA), the most common inherited cause of infant mortality. Even though the SMA phenotype is traditionally considered as related to spinal motor neuron loss, it ...
Paolo d'Errico +9 more
doaj +1 more source
Progranulin contributes to endogenous mechanisms of pain defense after nerve injury in mice [PDF]
, 2012 Progranulin haploinsufficiency is associated with frontotemporal dementia in humans. Deficiency of progranulin led to exaggerated inflammation and premature aging in mice.
Albuquerque, Boris +5 more
core +1 more source
Differential NPY-Y1 Receptor Density in the Motor Cortex of ALS Patients and Familial Model of ALS
Brain Sciences, 2021 Destabilization of faciliatory and inhibitory circuits is an important feature of corticomotor pathology in amyotrophic lateral sclerosis (ALS). While GABAergic inputs to upper motor neurons are reduced in models of the disease, less understood is the ...
Courtney M. Clark +5 more
doaj +1 more source
Translatomic analysis of regenerating and degenerating spinal motor neurons in injury and ALS
iScience, 2021 Summary: The neuromuscular junction is a synapse critical for muscle strength and coordinated motor function. Unlike CNS injuries, motor neurons mount robust regenerative responses after peripheral nerve injuries.
Jennifer L. Shadrach +6 more
doaj
Frontiers in Cellular Neuroscience, 2016 Spinal muscular atrophy (SMA) is an inherited neuromuscular disease primarily characterized by degeneration of spinal motor neurons, and caused by reduced levels of the SMN protein. Previous studies to understand the proteomic consequences of reduced SMN
Heidi R Fuller +12 more
doaj +1 more source
Acta Neuropathologica Communications, 2020 DNA damage is implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). However, relationships between DNA damage accumulation, DNA damage response (DDR), and upper and lower motor neuron vulnerability in human ALS are unclear; furthermore ...
Byung Woo Kim +3 more
doaj +1 more source
Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons. [PDF]
PLoS ONE, 2012 TDP-43 pathology in spinal motor neurons is a neuropathological hallmark of sporadic amyotrophic lateral sclerosis (ALS) and has recently been shown to be closely associated with the downregulation of an RNA editing enzyme called adenosine deaminase ...
Takuto Hideyama +4 more
doaj +1 more source
Identification of a rhythmic firing pattern in the enteric nervous system that generates rhythmic electrical activity in smooth muscle [PDF]
, 2018 The enteric nervous system (ENS) contains millions of neurons essential for organization of motor behavior of the intestine. It is well established that the large intestine requires ENS activity to drive propulsive motor behaviors.
Brookes, Simon J +10 more
core +2 more sources
Motor Neurons that Multitask [PDF]
Neuron, 2012 Animals use a form of sensory feedback termed proprioception to monitor their body position and modify the motor programs that control movement. In this issue of Neuron, Wen et al. (2012) provide evidence that a subset of motor neurons function as proprioceptors in C.
openaire +3 more sources
Direct conversion of human pluripotent stem cells into cranial motor neurons using a piggyBac vector [PDF]
, 2018 Human pluripotent stem cells (PSCs) are widely used for in vitro disease modeling. One of the challenges in the field is represented by the ability of converting human PSCs into specific disease-relevant cell types.
De Santis, Riccardo +5 more
core +2 more sources