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Статті в журналах з теми "SOD1G93A mouse model"
Kirby, Alastair J., Thomas Palmer, Richard J. Mead, Ronaldo M. Ichiyama, and Samit Chakrabarty. "Caudal–Rostral Progression of Alpha Motoneuron Degeneration in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis." Antioxidants 11, no. 5 (May 17, 2022): 983. http://dx.doi.org/10.3390/antiox11050983.
Повний текст джерелаBonifacino, Tiziana, Claudia Rebosio, Francesca Provenzano, Carola Torazza, Matilde Balbi, Marco Milanese, Luca Raiteri, Cesare Usai, Ernesto Fedele, and Giambattista Bonanno. "Enhanced Function and Overexpression of Metabotropic Glutamate Receptors 1 and 5 in the Spinal Cord of the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis during Disease Progression." International Journal of Molecular Sciences 20, no. 18 (September 13, 2019): 4552. http://dx.doi.org/10.3390/ijms20184552.
Повний текст джерелаMartin, Elodie, William Cazenave, Anne-Emilie Allain, Daniel Cattaert, and Pascal Branchereau. "Implication of 5-HT in the Dysregulation of Chloride Homeostasis in Prenatal Spinal Motoneurons from the G93A Mouse Model of Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 21, no. 3 (February 7, 2020): 1107. http://dx.doi.org/10.3390/ijms21031107.
Повний текст джерелаRudnick, Noam D., Christopher J. Griffey, Paolo Guarnieri, Valeria Gerbino, Xueyong Wang, Jason A. Piersaint, Juan Carlos Tapia, Mark M. Rich, and Tom Maniatis. "Distinct roles for motor neuron autophagy early and late in the SOD1G93A mouse model of ALS." Proceedings of the National Academy of Sciences 114, no. 39 (September 13, 2017): E8294—E8303. http://dx.doi.org/10.1073/pnas.1704294114.
Повний текст джерелаChiu, Isaac M., Adam Chen, Yi Zheng, Bela Kosaras, Stefanos A. Tsiftsoglou, Timothy K. Vartanian, Robert H. Brown, and Michael C. Carroll. "T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS." Proceedings of the National Academy of Sciences 105, no. 46 (November 7, 2008): 17913–18. http://dx.doi.org/10.1073/pnas.0804610105.
Повний текст джерелаTankersley, Clarke G., Christine Haenggeli, and Jeffery D. Rothstein. "Respiratory impairment in a mouse model of amyotrophic lateral sclerosis." Journal of Applied Physiology 102, no. 3 (March 2007): 926–32. http://dx.doi.org/10.1152/japplphysiol.00193.2006.
Повний текст джерелаMoreno-Martinez, Laura, Miriam de la Torre, María J. Muñoz, Pilar Zaragoza, José Aguilera, Ana C. Calvo, and Rosario Osta. "Neuroprotective Fragment C of Tetanus Toxin Modulates IL-6 in an ALS Mouse Model." Toxins 12, no. 5 (May 17, 2020): 330. http://dx.doi.org/10.3390/toxins12050330.
Повний текст джерелаRojas, Pilar, Ana I. Ramírez, Manuel Cadena, José A. Fernández-Albarral, Elena Salobrar-García, Inés López-Cuenca, Irene Santos-García, et al. "Retinal Ganglion Cell Loss and Microglial Activation in a SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 22, no. 4 (February 7, 2021): 1663. http://dx.doi.org/10.3390/ijms22041663.
Повний текст джерелаMallozzi, Cinzia, Alida Spalloni, Patrizia Longone, and Maria Rosaria Domenici. "Activation of Phosphotyrosine-Mediated Signaling Pathways in the Cortex and Spinal Cord of SOD1G93A, a Mouse Model of Familial Amyotrophic Lateral Sclerosis." Neural Plasticity 2018 (August 5, 2018): 1–10. http://dx.doi.org/10.1155/2018/2430193.
Повний текст джерелаRei, Nádia, Cláudia A. Valente, Sandra H. Vaz, Miguel Farinha-Ferreira, Joaquim A. Ribeiro, and Ana M. Sebastião. "Changes in adenosine receptors and neurotrophic factors in the SOD1G93A mouse model of amyotrophic lateral sclerosis: Modulation by chronic caffeine." PLOS ONE 17, no. 12 (December 14, 2022): e0272104. http://dx.doi.org/10.1371/journal.pone.0272104.
Повний текст джерелаДисертації з теми "SOD1G93A mouse model"
Li, Liang. "The role of calpain-calpastatin system in in the SOD1G93A mouse model of amyotrophic lateral sclerosis." Thesis, University of Brighton, 2009. https://research.brighton.ac.uk/en/studentTheses/e0f03b52-5ddc-42cc-b769-ccde3e69e709.
Повний текст джерелаMurphy, Matthew M. "Investigating the Effects of CyPPA on Small-Conductance Calcium-Activated Potassium Channels in SOD1G93A Transgenic Mouse Model." Wright State University / OhioLINK, 2020. http://rave.ohiolink.edu/etdc/view?acc_num=wright1589929963490428.
Повний текст джерелаTORAZZA, CAROLA. "Metabotropic glutamate receptor 5 as a target for the modulation of the reactive astrocyte phenotype in the SOD1G93A mouse model of amyotrophic lateral sclerosis." Doctoral thesis, Università degli studi di Genova, 2021. http://hdl.handle.net/11567/1044418.
Повний текст джерелаZhu, Hongmei. "Prenatal dysfunctions of chloride-related inhibition in lumbar motoneurons of the SOD1G93A ALS." Electronic Thesis or Diss., Bordeaux, 2023. http://www.theses.fr/2023BORD0026.
Повний текст джерелаAmyotrophic lateral sclerosis (ALS) is a fatal and adult-onset neurodegenerative disease characterized by a progressive degeneration of motoneurons (MNs) with complex multifactorial aetiology. Most ALS studies have focused on symptomatic stages based on the hypothesis that ALS pathogenesis occurs when the disease becomes symptomatic. However, growing evidence indicates that ALS pathogenesis might start long before symptom onset. My PhD thesis work was based on the hypothesis that ALS - familial and sporadic - stems from deficits taking place during early development. With the aim of identifying early changes underpinning ALS neurodegeneration, the first part of my thesis analysed the GABAergic/glycinergic inhibitory postsynaptic currents (IPSCs) to embryonic (E) E17.5 MNs located in the ventro-lateral motor column from SOD1G93A (SOD) mice, in parallel with the analyse of chloride homeostasis. Our results showed that IPSCs are less frequent in SOD animals in accordance with a reduction of synaptic VIAAT-positive terminals in the close proximity of MN somata. SOD MNs exhibited an ECI 10 mV more depolarized than wild type (WT) MNs. This deficit in GABA/glycine inhibition was due to a reduction of the neuronal chloride transporter KCC2. SOD spontaneous IPSCs and evoked GABAAR-currents exhibited a slower decay correlated to elevated [Cl-]i. Using computer modelling approach, we revealed that the slower relaxation of synaptic inhibitory events acts as a compensatory mechanism to strengthen or increase the efficacy of GABA/glycine inhibition when ECI is more depolarized. Interestingly, simulations revealed an excitatory effect of low frequency (<50Hz) depolarizing GABA/glycine post-synaptic potentials (dGPSPs) in SOD-like MNs but not in WT-like littermates. At high frequency, dGPSPs switched to inhibitory effect resulting from the summation of the shunting components. The second part of my PhD thesis focussed on the effect of electrically evoked-dGPSPs, at different frequencies (7.5 to 100 Hz), on real lumbar E17.5 MNs in which a depolarized ECI (below spike threshold) was imposed. The aim was to examine whether the excitatory effect could be linked to morphological changes previously described in E17.5 SOD MNs. Results showed that some MNs were excited by low frequency dGPSPs and inhibited by high frequency dGPSPs (Dual MNs) and others were inhibited at all frequencies (Inhibited MNs). Dual effect was more often detected in SOD MNs. WT MNs were classified into two clusters according to their input resistance (Rin), Dual MNs being specific to high Rin and Inhibited MNs to low Rin. Morphometric data pointed out a reduced dendritic tree in high Rin WT Dual MNs and a large dendritic tree in low Rin Inhibited MNs. This was not the case in SOD MNs that were excited or inhibited whatever their morphology and Rin. In agreement with simulation showing that a less density of inhibitory current on MNs soma favours excitatory dGPSPs, we found less synaptic VIAAT terminals on the soma and proximal dendrites of SOD MNs, compared to littermate WT MNs, as well as a lower frequency of spontaneous dGPSPs. Altogether, my thesis data emphasize a prenatal defect in the CI- homeostasis and GABA/glycine innervation in the SOD1G93A ALS MNs. Before birth, a dominant population of MNs with low Rin emerges in WT animals. These MNs that are inhibited by dGPSPs could represent future ALS vulnerable fast MNs (putative FF). Interestingly, those MNs are not inhibited in SOD animals. The inhibitory dysfunction could be attributed to two distinct factors: morphology and perisomatic inhibitory synapse density. Of these two factors, the latter plays a major role by controlling capability of GABAergic/glycinergic neurons for shaping spinal motor output
Santos, Catarina de Pais Paiva. "Glia-Motoneuron dialogue in ALS onset and progression in SOD1G93A-mice model." Master's thesis, 2014. http://hdl.handle.net/10362/13877.
Повний текст джерелаFundação para a Ciência e a Tecnologia - PTDC/SAU-FAR/118787/2010 (DB) and PEst-OE/SAU/UI4013/2013-2014
Fernandes, Filipe Jorge do Nascimento Xavier 1989. "Neuromuscular transmission modulation by A2A adenosine receptors in the SOD1G93A mouse model of amyotrophic lateral sclerosis." Master's thesis, 2013. http://hdl.handle.net/10451/10103.
Повний текст джерелаAmyotrophic Lateral Sclerosis (ALS) is an adult-onset progressive neurodegenerative disease characterized by the selective loss of motor neuron function leading to muscle atrophy and weakness. After symptoms onset patients last 4 to 5 years and ultimately die due to bulbar failure. Animal models have been developed to study the neurobiology of ALS, with the SOD1G93A mouse model as the most studied so far. Morphological and functional abnormalities have been reported in both pre-symptomatic and symptomatic stages of ALS progression in this rodent model. Dysfunctions in neuromuscular transmission at phrenic nerve-hemidiaphragm preparations of the SOD1G93A mouse have recently been put in evidence by our group. Since adenosine A2A receptors (A2AR) play a major role in fine-tuning neurotransmitter release in mammalian neuromuscular junctions (NMJs), we decided to evaluate how A2AR modulate acetylcholine (ACh) release in presymptomatic (4-6 weeks old (wo)) and symptomatic (12-14 wo) SOD1G93A mice. Using the selective A2AR agonist CGS 21680, we performed a dose-response study using 3, 5 and 10 nM in pre-symptomatic SOD1G93A mice and age-matched Wild Type (WT) animals. CGS 21680 at 3, 5, 10 and 25 nM was studied in symptomatic SOD1G93A and 12-14 wo WT rodents. 25 nM was used in the previous mentioned group of animals to verify if at concentrations higher than 10nM CGS 21680 effect in NMT would be increased. Intracellular recordings of endplate potentials (EPPs), miniature endplate potentials (MEPPs) and giant miniature endplate potentials (GMEPPs) where performed in high [Mg2+] paralyzed phrenic nerve-hemidiaphragm preparations. Low-frequency stimuli (0.5 Hz) allowed the evaluation of the evoked activity (EPPs) while spontaneous activity (MEPPs and GMEPPs) was measured in gap-free intervals. Quantal content (QC) was measured as the ratio between the mean EPP amplitude and the mean MEPP amplitude recorded in the same period of time. We first validated high [Mg2+] as an useful method to study neuromuscular transmission (NMT) in these mice. The shift in the Ca2+/Mg2+ ratio preserved the previous described features of ACh release in SOD1G93A mice diaphragm fibers. In the pre-symptomatic phase, SOD1G93A mice displayed a significant increase in EPP amplitude and QC in tested concentrations of CGS 21680 when compared to WT mice (p<0.05 Unpaired t-test) except for EPP changes at 3nM (p>0.05 Unpaired t-test). MEPP and GMEPP amplitude were not changed by CGS 21680 (p>0.05 Paired t-test). The A2AR-mediated increase in MEPP frequency was not statistically different between both groups in the presence of 3 and 10nM of the A2AR agonist (p>0.05 Unpaired t-test), but was significantly higher in SOD1G93A mice when perfused at 5nM (p<0.05 Unpaired t-test). We also found that in the pre-symptomatic SOD1G93A fibers GMEPP frequency was statistically higher in the presence of CGS 21680 (5nM) than in age-matched WT NMJs (p<0.05 Unpaired t-test). In the symptomatic phase, CGS 21680 did not elicit any changes in SOD1G93A mice evoked (EPP amplitude and QC) and spontaneous (MEPP and GMEPP frequency and amplitude) activity from SOD1G93A mice (p>0.05 Paired t-test). When 0.5 Hz stimuli were delivered, EPP amplitude, QC and MEPP frequency were increased in 12-14 wo WT mice in the presence of 3, 5, 10 and 25 nM of CGS 21680 (p<0.05 Paired t-test). These parameters together with GMEPP frequency were significantly higher than in symptomatic mice (p<0.05 Unpaired t-test). GMEPP and MEPP amplitude were not changed by CGS 21680 in older WT mice (p>0.05 Paired t-test). The A2AR receptor antagonist SCH 58261 (50nM) was devoid of effect in spontaneous and evoked release (p>0.05 Paired t-test) and effectively blocked CGS 21680 (5nM in 4-6 wo mice and 10 nM in 12-14 wo WT rodents) facilitation (p<0.05 one-way ANOVA followed by Tukey’s pos-hoc). Our results strongly suggest an early functionally enhancement of NMT concerning adenosine modulation before symptoms appear. This dysfunction involves an A2AR functional upregulation at diaphragm NMJs in pre-symptomatic SOD1G93A mice. When symptoms start to develop, A2AR receptor function is lost in symptomatic SOD1G93A mice but remains present in 12-14 wo WT mice. Furthermore, CGS 21680 perfusion triggered a higher A2AR facilitation in 12-14 wo than in 4-6 wo WT mice. This might be attributable to a normal maturation feature of the studied synapse, which contrasted with the observations in transgenic mice. The shift from a functional upregulation of A2AR before symptomatology that arises in SOD1G93A mice towards an apparent loss of A2AR functionality in symptomatic phase, highlights the role of this subtype of P1 receptors in the scope of ALS. A2AR targeted drugs could eventually play an import role in the delay of this disease.
A Esclerose Lateral Amiotrófica (ALS) é uma doença neurodegenerativa progressiva caracterizada pela perda seletiva da função do neurónio motor, levando a atrofia e fraqueza muscular. Após o início dos sintomas a esperança de vida dos doentes é de cerca de 4 a 5 anos, sendo a causa da morte geralmente devida a insuficiência respiratória. Modelos animais têm sido desenvolvidos para estudar a neurobiologia da ALS, sendo o do murganho SOD1G93A o mais estudado. Neste modelo, anormalidades morfológicas e funcionais têm sido relatadas em ambos os estadios pré-sintomático e sintomático da ALS. Disfunções na transmissão neuromuscular (NMT) em preparações de nervo frénico-hemidiafragma de murganho SOD1G93A foram recentemente postas em evidência pelo nosso grupo. Considerando que os recetores A2A de adenosina (A2AR) desempenham um papel importante no controlo da libertação de acetilcolina (ACh) na junção neuromuscular (NMJ) de mamífero, explorámos o papel dos A2AR na modulação da NMT em murganhos SOD1G93A nas fases pré-sintomática (4-6 semanas de idade) e sintomática (12-14 semanas de idade). Usando o agonista seletivo dos A2AR (CGS 21680), foi realizado um estudo de dose-resposta utilizando concentrações de 3, 5 e 10 nM em animais pré-sintomáticos SOD1G93A e Wild Type (WT) (4-6 semanas). CGS 21680 a 3, 5, 10 e 25 nM foi utilizado em murganhos sintomáticos SOD1G93A e WT (12 - 14 semanas). A concentração 25 nM foi testada neste último grupo, de modo a verificar se a concentrações superiores a 10 nM o efeito modulador do CGS 21680 na NMT estaria aumentado. Foram realizados registos intracelulares de potenciais de placa motora (EPPs), potenciais miniatura de placa motora (MEPPs) e potenciais miniatura gigantes de placa motora (GMEPPs) em preparações de nervo frénico-hemidiafragma paralisadas com elevada [Mg2+]. Estímulos de baixa frequência (0.5 Hz) permitiram a avaliação da atividade evocada (EPPs), ao passo que as atividades espontâneas (MEPPs e GMEPPs) foram medidas sem estimulação elétrica. O conteúdo quântico (QC) foi considerado como sendo a razão entre a média da amplitude dos EPPs e média da amplitude dos MEPPs registados no mesmo período de tempo. Inicialmente validámos o aumento da [Mg2+] como um método útil para o estudo da NMT nos murganhos SOD1G93A. A alteração do equilíbrio entre a razão Ca2 +/ Mg2+ preservou as características da NMT anteriormente descritas neste modelo. Na fase pré-sintomática, os animais SOD1G93A apresentaram um aumento na amplitude do EPP e do QC nas concentrações testadas de CGS 21680 quando comparado com murganhos WT (p<0.05 Unpaired t-test), exceto nas variações do EPP a 3nM (p>0.05 Unpaired t-test). A amplitude dos MEPPs e GMEPPs não foram alteradas pelo CGS 21680 (p>0.05 Paired t-test). O aumento na frequência dos MEPPs resultante da ativação dos A2AR não foi significativamente diferente entre os dois grupos na presença de 3 e 10 nM do agonista dos A2AR (p>0.05 Unpaired t-test) mas foi estatisticamente superior em murganhos SOD1G93A quando perfundido a 5nM (p<0.05 Unpaired t-test). As NMJs dos animais SOD1G93A pré-sintomáticos apresentaram aumento na frequência dos GMEPPs na presença de CGS 21680 (5 nM) quando comparadas com as dos animais WT (p<0.05 Unpaired t-test). Na fase sintomática, o CGS 21680 não alterou a resposta evocada (amplitude dos EPP e QC) nem a atividade espontânea (frequência e amplitude dos MEPPs e GMEPPs) dos animais SOD1G93A (p>0.05 Paired t-test). Após estimulação de 0.5 Hz a amplitude dos EPPs, QC e frequência dos MEPPs aumentaram em murganhos WT na presença de 3, 5, 10 e 25 nM de CGS 21680 (p<0.05 Paired t-test). Estes parâmetros, juntamente com frequência dos GMEPPs foram significativamente mais elevados do que em murganhos sintomáticos (p<0,05 Unpaired t-test). A amplitude dos GMEPPs e MEPPs não foram alteradas pela aplicação de CGS 21680 em murganhos WT (p>0.05 Paired t-test). O antagonista dos A2AR SCH 58261 (50nM) não alterou a actividade espontânea e evocada (p>0.05 Paired t-test) mas bloqueou eficazmente o efeito facilitatório do CGS 21680 (a 5nM em animais de 4-6 semanas e a 10nM em murganhos WT com 12-14 semanas) (p<0.05 Unpaired t-test). Os resultados deste estudo apontam para a existência de uma disfunção da modulação adenosinérgica na libertação de ACh antes do aparecimento dos sintomas. Esta disfunção envolve uma sobrerregulação funcional dos A2AR nas NMJs de diafragma de murganhos SOD1G93A pré-sintomáticos. Quando os sintomas começam a aparecer, a função dos A2AR parece desaparecer em murganhos sintomáticos, mas permanece presente em animais WT de 12-14 semanas de idade. Além disso, o CGS 21680 promoveu uma facilitação A2AR superior em murganhos WT de 12-14 semanas do que de 4-6 semanas. Isto pode ser atribuído a uma característica normal de maturação da sinapse, contrastando com as observações em animais SOD1G93A. A passagem de uma sobrerregulação funcional dos A2AR antes da sintomatologia surgir para uma aparente perda da funcionalidade dos A2AR durante a fase sintomática em animais SOD1G93A, destaca o papel deste subtipo de recetores P1 no âmbito da ALS. Tendo isto em consideração, fármacos que atuem nos A2AR poderão eventualmente desempenhar um papel importante no atraso desta doença.
Частини книг з теми "SOD1G93A mouse model"
Puig, Cristina, Ana Fernández, Luis Gandía, and Antonio G. García. "The amyotrophic lateral sclerosis SOD1G93A mouse model and applications: Studies into the P2X7 receptor." In Handbook of Animal Models in Neurological Disorders, 81–94. Elsevier, 2023. http://dx.doi.org/10.1016/b978-0-323-89833-1.00048-3.
Повний текст джерелаТези доповідей конференцій з теми "SOD1G93A mouse model"
Kim, Ahwon, Do-Yeon Lee, and Jung-Joon Sung. "Cdk5 inhibition in the SOD1G93A transgenic mouse model of amyotrophic lateral sclerosis suppresses neurodegeneration and extends survival. (P1-6.001)." In 2023 Annual Meeting Abstracts. Lippincott Williams & Wilkins, 2023. http://dx.doi.org/10.1212/wnl.0000000000202934.
Повний текст джерела