Results 31 to 40 of about 1,754,547 (304)

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, Marina Boido, Antonio Piras, Valeria Valsecchi, Elena De Amicis, Denise Locatelli, Silvia Capra, Francesco Vagni, Alessandro Vercelli, Giorgio Battaglia   +9 more
doaj   +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, Rosemary M. Clark, Joshua A. Hoyle, Jyoti A. Chuckowree, Catriona A. McLean, Tracey C. Dickson   +5 more
doaj   +1 more source

Selective gating of neuronal activity by intrinsic properties in distinct motor rhythms [PDF]

, 2015
This research has been supported by the Royal Society, Wellcome Trust (089319), and the Biotechnology and Biological Sciences Research Council (BB/L0011X/1). I thank Drs.
Li, Wen-Chang, Li, Wen-Chang; id_orcid
core   +1 more source

DNA damage accumulates and responses are engaged in human ALS brain and spinal motor neurons and DNA repair is activatable in iPSC-derived motor neurons with SOD1 mutations

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, Ye Eun Jeong, Margaret Wong, Lee J. Martin   +3 more
doaj   +1 more source

Scaling proprioceptor gene transcription by retrograde NT3 signaling [PDF]

, 2011
The assembly of neuronal circuits depends critically on the sequential activation of transcriptional programs in defined neuronal sub-populations. In the spinal cord, retrograde signaling interactions from the periphery have been shown to be essential ...
Lee, Jun
core   +1 more source

Calcitonin gene-related peptide expression levels predict motor neuron vulnerability in the superoxide dismutase 1-G93A mouse model of amyotrophic lateral sclerosis

Neurobiology of Disease, 2012
In amyotrophic lateral sclerosis (ALS) some motor neurons degenerate while others survive. The molecular mechanisms underlying this selective vulnerability and resistance, respectively, are poorly understood.
Cornelia Ringer, Eberhard Weihe, Burkhard Schütz   +2 more
doaj   +1 more source

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

Cell Reports, 2023
Summary: 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 ...
Peter Harley, Caoimhe Kerins, Ariana Gatt, Guilherme Neves, Federica Riccio, Carolina Barcellos Machado, Aimee Cheesbrough, Lea R’Bibo, Juan Burrone, Ivo Lieberam   +9 more
doaj   +1 more source

Functional modulation of IFT kinesins extends the sensory repertoire of ciliated neurons in Caenorhabditis elegans [PDF]

, 2006
The diversity of sensory cilia on Caenorhabditis elegans neurons allows the animal to detect a variety of sensory stimuli. Sensory cilia are assembled by intraflagellar transport (IFT) kinesins, which transport ciliary precursors, bound to IFT particles,
James E. Evans, Kent L. McDonald, Amy L. Gunnarson, Gunnarson, Amy L., Guangshuo Ou, Snow, JJ, McDonald, KL, Stahlberg, Henning, Ou, Guangshuo, Ou, GS, Evans, JE, Stahlberg, H, Evans, James E., Jonathan M. Scholey, Scholey, JM, Gunnarson, AL, McDonald, Kent L., Joshua J. Snow, Henning Stahlberg, Snow, Joshua J., Scholey, Jonathan M.   +20 more
core   +1 more source

Motor Neurone Disease [PDF]

Postgraduate Medical Journal, 1962
THE story of motor neurone disease goes back more than a hundred years, to the latter half of the igth century, and the days of the great clinical neurologists of France. Of the many famous names linked to this story three are pre-eminent: Charcot-physician and neuropathologist, and great teacher, who became even more renowned for his studies of ...
openaire   +2 more sources

fMRI evidence of ‘mirror’ responses to geometric shapes [PDF]

, 2012
Mirror neurons may be a genetic adaptation for social interaction [1]. Alternatively, the associative hypothesis [2], [3] proposes that the development of mirror neurons is driven by sensorimotor learning, and that, given suitable experience, mirror ...
Heyes, C., Heyes Cecilia, Widmann, H, Clare Press, Press, C., Bird Geoffrey, Catmur, C, Press, C, Heyes, C, Cook, R, Bird, G., Cecilia Heyes, Cook Richard, Geoffrey Bird, Press Clare, Avenanti, A., Widmann Hannah, Heyes, Cecilia, Richard Cook, Bird, Geoffrey; id_orcid, Cook, R., Catmur, Caroline; id_orcid, Widmann, Hannah, Hannah Widmann, Bird, G, Widmann, H., Bird, Geoffrey, Heyes, Cecelia, Catmur, C., Catmur Caroline, Clare Press, Cook, Richard; id_orcid, Press, Clare, Caroline Catmur   +33 more
core   +2 more sources