Results 41 to 50 of about 73,691 (271)

Pharmacokinetics of therapies approved for spinal muscular atrophy: A narrative review of current evidence

Journal of International Medical Research
Spinal muscular atrophy is a severe neuromuscular disorder caused by mutations in the survival motor neuron 1 gene, leading to progressive motor neuron degeneration.
Eda Kübra Sel, Elvira Meni Maria Gkrinia, Rossana Roncato, Dinko Vitezić, Slobodan Janković, Andrej Belančić,   +6 more
doaj   +1 more source

Reciprocal control of viral infection and phosphoinositide dynamics

FEBS Letters, EarlyView.
Phosphoinositides, although scarce, regulate key cellular processes, including membrane dynamics and signaling. Viruses exploit these lipids to support their entry, replication, assembly, and egress. The central role of phosphoinositides in infection highlights phosphoinositide metabolism as a promising antiviral target.
Marie Déborah Bancilhon, Bruno Mesmin
wiley   +1 more source

Organ‐specific redox imbalances in spinal muscular atrophy mice are partially rescued by SMN antisense oligonucleotides

FEBS Letters, EarlyView.
We identified a systemic, progressive loss of protein S‐glutathionylation—detected by nonreducing western blotting—alongside dysregulation of glutathione‐cycle enzymes in both neuronal and peripheral tissues of Taiwanese SMA mice. These alterations were partially rescued by SMN antisense oligonucleotide therapy, revealing persistent redox imbalance as ...
Sofia Vrettou, Brunhilde Wirth
wiley   +1 more source

AAV-based delivery of RNAi targeting ataxin-2 improves survival and pathology in TDP-43 mice

Nature Communications
Amyotrophic lateral sclerosis (ALS) involves motor neuron death due to mislocalized TDP-43. Pathologic TDP-43 associates with stress granules (SGs), and lowering the SG-associated protein ataxin-2 (ATXN2) using Atxn2-targeting antisense oligonucleotides ...
Defne A. Amado, Ashley B. Robbins, Katherine R. Whiteman, Alicia R. Smith, Guillem Chillon, Yonghong Chen, Joshua A. Fuller, Nicholas A. Patty, Aleksandar Izda, Congsheng Cheng, Shareen Nelson, Abigail I. Dichter, Esteban O. Mazzoni, Alex Mas Monteys, Beverly L. Davidson   +14 more
doaj   +1 more source

Evaluation of potential effects of Plastin 3 overexpression and low-dose SMN-antisense oligonucleotides on putative biomarkers in spinal muscular atrophy mice. [PDF]

PLoS ONE, 2018
OBJECTIVES:Spinal muscular atrophy (SMA) is a devastating motor neuron disorder caused by homozygous loss of the survival motor neuron 1 (SMN1) gene and insufficient functional SMN protein produced by the SMN2 copy gene.
Eike A Strathmann, Miriam Peters, Seyyedmohsen Hosseinibarkooie, Frank W Rigo, C Frank Bennett, Phillip G Zaworski, Karen S Chen, Michael Nothnagel, Brunhilde Wirth   +8 more
doaj   +1 more source

Seasonal changes in patterns of gene expression in avian song control brain regions [PDF]

, 2012
Photoperiod and hormonal cues drive dramatic seasonal changes in structure and function of the avian song control system. Little is known, however, about the patterns of gene expression associated with seasonal changes.
Thompson, Christopher K, David F Clayton, Wissman, AM, Christopher K. Thompson (170958), Karin L Lent, Meitzen, John, John Meitzen, Replogle, K, Theo K. Bammler (128417), Brenowitz, Eliot A, Eliot A. Brenowitz (170987), Clayton, David F, Wissman, Anne Marie, Lent, KL, Farin Federico M., Lent Karin L., David J Perkel, Perkel David J., Richard P Beyer, Clayton, DF, Kirstin Replogle (46753), Kirstin Replogle, Meitzen, J, Bammler, TK, Farin, FM, Thompson Christopher K., Federico M. Farin (128418), Beyer, Richard P, Perkel, David J, David F. Clayton (170979), Beyer, RP, Anne Marie Wissman (170971), Wissman Anne Marie, Drnevich, J, Thompson, CK, Replogle Kirstin, Christopher K Thompson, Bammler Theo K., Federico M Farin, Meitzen John, Eliot A Brenowitz, Jenny Drnevich, Bammler, Theo K, Drnevich Jenny, John Meitzen (170962), Replogle, Kirstin, Farin, Federico M, Brenowitz, EA, Richard P. Beyer (128416), Anne Marie Wissman, Drnevich, Jenny, Karin L. Lent (170969), Theo K Bammler, Beyer Richard P., Lent, Karin L, Clayton David F., Brenowitz Eliot A., Perkel, DJ, David J. Perkel (170983), Jenny Drnevich (170966)   +59 more
core   +1 more source

Diversity and complexity in neural organoids

FEBS 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

Pro-inflammatory interleukin-18 increases Alzheimer’s disease-associated amyloid-β production in human neuron-like cells [PDF]

, 2012
Background: Alzheimer’s disease (AD) involves increased accumulation of amyloid-β (Aβ) plaques and neurofibrillary tangles as well as neuronal loss in various regions of the neocortex.
Anderson, G., Anderson George, Ojala, J.O., Sutinen, E.M., Pirttilä Tuula, Antero Salminen, Salminen Antero, Johanna O Ojala, Salminen, A., Elina M Sutinen, George Anderson, Tuula Pirttilä, Pirttilä, T., Sutinen Elina M, Ojala Johanna O   +14 more
core   +1 more source

Microbiome−host proteostasis crosstalk—An emerging perspective on mechanisms and interventions toward healthy longevity

FEBS Letters, EarlyView.
Proteostasis and the gut microbiota play a key role in shaping host physiology. Microbiota‐derived metabolites, vitamins, and RNA modulate host proteostasis. Findings from model systems, including C. elegans, indicate microbes can either stabilize or disrupt host proteostasis.
Abhishek Anil Dubey, Maria Ermolaeva
wiley   +1 more source

Survival motor neuron (SMN) protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis [PDF]

, 2015
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease involving mainly the upper and lower motor neurons of adult humans. With regard to the pathomechanism of spinal anterior horn cell (AHC) degeneration in ALS, copy number ...
Takahama, Sachiko, Kakita, Akiyoshi, Takahashi, Hitoshi, Mizutani, Toshio, Komori, Takashi, Piao, Yingshan, Morita, Takashi, Hashimoto, Tomoyo, Oyanagi, Kiyomitsu   +8 more
core   +2 more sources