Molecular determinants of signal transduction in tropomyosin receptor kinases
Tropomyosin receptor kinases control critical neuronal functions, but how do the same receptors produce diverse cellular responses? This review explores the structural mechanisms behind Trk signaling diversity, focusing on allosteric modulation and ligand bias.
Giray Enkavi
wiley +1 more source
Profound downregulation of the RNA editing enzyme ADAR2 in ALS spinal motor neurons
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset fatal motor neuron disease. In spinal motor neurons of patients with sporadic ALS, normal RNA editing of GluA2, a subunit of the L-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid ...
Takuto Hideyama +6 more
doaj +1 more source
MFN2 Couples Glutamate Excitotoxicity and Mitochondrial Dysfunction in Motor Neurons*♦
Background: Mitochondrial function is dependent on mitochondrial fission and fusion dynamics. Results: Calpain-mediated degradation of MFN2 is responsible for glutamate-induced mitochondrial dysfunction and neuronal death in spinal cord motor neurons ...
Wenzhang Wang +9 more
semanticscholar +1 more source
Naked cuticle is essential for Drosophila wing development beyond Wingless signaling
Naked cuticle (Nkd), a Wnt signaling inhibitor, assumes extensive roles in Drosophila wing development. Overexpressing Nkd causes smaller, crumpled wings, while also perturbing multiple signaling pathways and developmental genes. A specific region (R1S) is critical for Nkd's function as a signaling integrator, offering new insights for studying its ...
Rui Wang, Ping Wang
wiley +1 more source
Modeling Motor Neuron Resilience in ALS Using Stem Cells
Summary: Oculomotor neurons, which regulate eye movement, are resilient to degeneration in the lethal motor neuron disease amyotrophic lateral sclerosis (ALS). It would be highly advantageous if motor neuron resilience could be modeled in vitro.
Ilary Allodi +8 more
doaj +1 more source
Spinal V2b neurons reveal a role for ipsilateral inhibition in speed control [PDF]
The spinal cord contains a diverse array of interneurons that govern motor output. Traditionally, models of spinal circuits have emphasized the role of inhibition in enforcing reciprocal alternation between left and right sides or flexors and extensors ...
Bagnall, Martha W +5 more
core +1 more source
Metamodulation of a spinal locomotor network by nitric oxide [PDF]
Flexibility in the output of spinal networks can be accomplished by the actions of neuromodulators; however, little is known about how the process of neuromodulation itself may be modulated.
McLean, DL, Sillar, Keith Thomas
core +1 more source
Metformin mediates mitochondrial quality control in Leber's hereditary optic neuropathy (LHON) fibroblasts carrying mtDNA mutations. At therapeutic levels, metformin activates AMPK signaling to restore mitochondrial dynamics by promoting fusion and restraining fission, while preserving mitochondrial mass, enhancing autophagy/mitophagy and biogenesis ...
Chatnapa Panusatid +3 more
wiley +1 more source
Nonsense mutations in alpha-II spectrin in three families with juvenile onset hereditary motor neuropathy [PDF]
Distal hereditary motor neuropathies are a rare subgroup of inherited peripheral neuropathies hallmarked by a length-dependent axonal degeneration of lower motor neurons without significant involvement of sensory neurons.
Asselbergh, B +10 more
core +1 more source
ALS IMPLICATED PROTEIN TDP-43 SUSTAINS LEVELS OF STMN2 A MEDIATOR OF MOTOR NEURON GROWTH AND REPAIR
The findings that amyotrophic lateral sclerosis (ALS) patients almost universally display pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its gene cause familial ALS have nominated altered RNA metabolism as a disease ...
Joseph R. Klim +15 more
semanticscholar +1 more source

