Results 71 to 80 of about 553,827 (305)

Removal of GABAA receptor γ2 subunits from parvalbumin neurons causes wide-ranging behavioral alterations [PDF]

open access: yes, 2011
We investigated the behavioral significance of fast synaptic inhibition by αβγ2-type GABA(A) receptors on parvalbumin (Pv) cells. The GABA(A) receptor γ2 subunit gene was selectively inactivated in Pv-positive neurons by Cre/loxP recombination.
Leppa, E.   +10 more
core   +1 more source

Valosin‐containing protein counteracts ATP‐driven dissolution of FUS condensates through its ATPase activity in vitro

open access: yesFEBS Letters, EarlyView.
Biomolecular condensates formed by fused in sarcoma (FUS) are dissolved by high ATP concentrations yet persist in cells. Using a reconstituted system, we demonstrate that valosin‐containing protein (VCP), an AAA+ ATPase, counteracts ATP‐driven dissolution of FUS condensates through its D2 ATPase activity.
Hitomi Kimura   +2 more
wiley   +1 more source

Increased motor neuron resilience by small molecule compounds that regulate IGF-II expression

open access: yesNeurobiology of Disease, 2018
The selective vulnerability of motor neurons in amyotrophic lateral sclerosis (ALS) is evident by sparing of a few subpopulations during this fast progressing and debilitating degenerative disease. By studying the gene expression profile of resilient vs.
Teresia M. Osborn   +2 more
doaj   +1 more source

Diversity and complexity in neural organoids

open access: yesFEBS Letters, EarlyView.
Neural organoid research aims to expand genetic diversity on one side and increase tissue complexity on the other. Chimeroids integrate multiple donor genomes within single organoids. Self‐organising multi‐identity organoids, exogenous cell seeding, or enforced assembly of region‐specific organoids contribute to tissue complexity.
Ilaria Chiaradia, Madeline A. Lancaster
wiley   +1 more source

The Timing and Extent of Motor Neuron Vulnerability in ALS Correlates with Accumulation of Misfolded SOD1 Protein in the Cortex and in the Spinal Cord

open access: yesCells, 2020
Understanding the cellular and molecular basis of selective vulnerability has been challenging, especially for motor neuron diseases. Developing drugs that improve the health of neurons that display selective vulnerability relies on in vivo cell-based ...
Baris Genc   +3 more
doaj   +1 more source

Sensory neurons do not induce motor neuron loss in a human stem cell model of spinal muscular atrophy. [PDF]

open access: yesPLoS ONE, 2014
Spinal muscular atrophy (SMA) is an autosomal recessive disorder leading to paralysis and early death due to reduced SMN protein. It is unclear why there is such a profound motor neuron loss, but recent evidence from fly and mouse studies indicate that ...
Andrew J Schwab, Allison D Ebert
doaj   +1 more source

Use of a recombinant pseudorabies virus to analyze motor cortical reorganization after unilateral facial denervation [PDF]

open access: yes, 2005
A unilateral facial nerve injury (n7x) was found to influence the transcallosal spread of the attenuated strain of pseudorabies virus (PRV Bartha) from the affected (left) primary motor cortex (MI) to the contralateral MI of rats.
Boda, Krisztina   +8 more
core   +1 more source

Diagnosing motor neurone disease [PDF]

open access: yesBMJ, 1996
Advanced motor neurone disease is easily recognised. Progressively worsening dysphagia and dysarthria, and widespread wasting and weakness of the limbs with hyperactive reflexes in a corresponding wasted myotome in the absence of other neurological signs are unmistakable features.
openaire   +2 more sources

Linking neurogenesis, oligodendrogenesis, and myelination defects to neurodevelopmental disruption in primary mitochondrial disorders

open access: yesFEBS Letters, EarlyView.
Mitochondrial remodeling shapes neural and glial lineage progression by matching metabolic supply with demand. Elevated OXPHOS supports differentiation and myelin formation, while myelin compaction lowers mitochondrial dependence, revealing mitochondria as key drivers of developmental energy adaptation.
Sahitya Ranjan Biswas   +3 more
wiley   +1 more source

Non-viral gene therapy that targets motor neurons in vivo

open access: yesFrontiers in Molecular Neuroscience, 2014
A major challenge in neurological gene therapy is safe delivery of transgenes to sufficient cell numbers from the circulation or periphery. This is particularly difficult for diseases involving spinal cord motor neurons such as amyotrophic lateral ...
Mary-Louise eRogers   +6 more
doaj   +1 more source

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