Article
Neurosciences
Wan-Ling Tsai, Chih-Ju Chang, Chih-Yang Wang, Tsung- Hsu, Ming-Yuan Chang, Yi-Hsin Wu, Pei-Shan Chang, Kai-Lun Lin, Jian-Ying Chuang, Artur Kania, Tzu-Jen Kao
Summary: The study demonstrates the essential role of paxillin in motor axon guidance in both chick and mouse embryos. Paxillin is involved in the Ephrin-Eph signaling pathway, influencing the trajectory selection of LMC axons.
JOURNAL OF NEUROSCIENCE
(2021)
Review
Biochemistry & Molecular Biology
Rachel James, Helena Chaytow, Leire M. Ledahawsky, Thomas H. Gillingwater
Summary: SMA is an autosomal recessive motor neuron disease caused by mutations in the SMN1 gene. The development of combinatorial therapies for SMA is necessary, with mitochondria being a relevant target for such therapies. Understanding mitochondrial dysfunction in SMA may lead to the development of targeted mitochondrial therapies with potential benefits for other motor neuron diseases and neurodegenerative disorders.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Article
Developmental Biology
Fumiyasu Imai, Mike Adam, S. Steven Potter, Yutaka Yoshida
Summary: The study reveals the crucial role of HoxD family transcription factors in the specificity of monosynaptic sensory-motor connections in the spinal cord. Mice lacking Hoxd9, Hoxd10 and Hoxd11 show locomotion defects and aberrant axon development in quadriceps motor neurons.
Article
Clinical Neurology
Nicolas Delestree, Evangelia Semizoglou, John G. Pagiazitis, Aleksandra Vukojicic, Estelle Drobac, Vasilissa Paushkin, George Z. Mentis
Summary: Serotonin plays a crucial role in regulating neuronal networks responsible for various essential behaviors. Dysfunction of serotonergic neurotransmission is found in spinal muscular atrophy (SMA), a neurodegenerative disease. The study shows severe dysfunction of serotonergic neuromodulation in SMA mice, suggesting that targeting 5-HT neuromodulation could be a potential therapeutic approach.
Article
Biology
Saul Bello-Rojas, Martha W. Bagnall
Summary: The study found that sister V2a/b neurons in the zebrafish spinal cord receive input from distinct presynaptic sources, do not communicate with each other, and connect to largely distinct targets.
Article
Neurosciences
Ryan W. Castro, Mikayla C. Lopes, Lindsay M. De Biase, Gregorio Valdez
Summary: Microglia display region-dependent deleterious features with age and diseases in the brain, and it remains unclear if similar phenotypic heterogeneity exists in the spinal cord. The study shows that regardless of regional location, spinal cord microglia become increasingly activated during aging. However, microglia in the ventral horn lose spatial organization and aggregate around motor neurons, potentially affecting motor neuron function.
Article
Clinical Neurology
M. Khamaysa, M. Lefort, M. Pelegrini-Issac, A. Lackmy-Vallee, A. Preuilh, D. Devos, A. -S. Rolland, C. Desnuelle, M. Chupin, V. Marchand-Pauvert, G. Querin, Pierre-Francois Pradat
Summary: This study aimed to evaluate the predictive value of cervical spinal cord MRI parameters for motor capacity in ALS compared to clinical prognostic factors. Structural MRI measurements were significantly correlated with the ALSFRS-R score and its sub-scores. Multiple linear regression models combining spinal multimodal MRI and clinical factors could predict motor capacity in ALS.
JOURNAL OF NEUROLOGY
(2023)
Article
Neurosciences
Zachary T. Olmsted, Cinzia Stigliano, Brandon Marzullo, Jose Cibelli, Philip J. Horner, Janet L. Paluh
Summary: This study evaluates the survival and integration of hiPSC-derived spinal motor neurons and oligodendrocyte progenitor cells in a rat model, demonstrating successful transplantation and meeting functional electrophysiology parameters. The findings provide a streamlined and predictable approach for neural cell-based therapies of spinal cord injury.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2022)
Article
Biology
Arnault H. Caillet, Andrew T. M. Phillips, Dario Farina, Luca Modenese
Summary: The study focuses on deriving mathematical relationships between MN electrophysiological and anatomical properties by reprocessing data from 40 experimental studies in adult cat, rat, and mouse MN preparations. The validated mathematical framework provides a convenient tool for neuroscientists and experimenters. By using this framework, modelers can build profiles of inter-consistent MN-specific properties to scale pools of MN models, enhancing the accuracy and interpretability of simulations.
Article
Neurosciences
Mariangeles Kovacs, Catalina Alamon, Cecilia Maciel, Valentina Varela, Sofia Ibarburu, Lucas Tarrago, Peter H. King, Ying Si, Yuri Kwon, Olivier Hermine, Luis Barbeito, Emiliano Trias
Summary: This study identified the presence of c-Kit+ MCs in the CNS of ALS patients and suggested a potential pathological mechanism triggered by these cells through an SCF/c-Kit-dependent mechanism. Pharmacological inhibition of c-Kit may reduce the number of MCs in ALS, providing a potential therapeutic target for the disease.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2021)
Article
Clinical Neurology
Linda-Isabell Schmitt, Christina David, Rebecca Steffen, Stefanie Hezel, Andreas Roos, Ulrike Schara-Schmidt, Christoph Kleinschnitz, Markus Leo, Tim Hagenacker
Summary: Spinal muscular atrophy (SMA) is a neurodegenerative disorder characterized by the loss of motor neurons (MNs) due to a genetic mutation. This study investigates the role of spinal astrocytes and the excitatory amino acid transporter 1 (EAAT1) in the pathogenesis of late-onset SMA. The results suggest that EAAT1 reduction leads to elevated levels of glutamate, causing glutamate excitotoxicity and subsequent loss of MNs.
ACTA NEUROPATHOLOGICA
(2023)
Review
Neurosciences
Giuliano Taccola, Ronaldo M. Ichiyama, V. Reggie Edgerton, Parag Gad
Summary: This review discusses the physiological states of spinal networks, highlighting the stochastic modulation by changing ensembles of proprioceptive and supraspinal input. Spinal epidural interfaces offer a platform for studying spinal network dynamics post-injury. Low-frequency epidural pulses can evoke motor responses with oscillatory patterns. The neural variability among spinal networks is seen as a fundamental mechanism of network design rather than noise interfering with movement control.
EXPERIMENTAL NEUROLOGY
(2022)
Review
Immunology
Yizhang Mo, Kebing Chen
Summary: High mobility group box 1 (HMGB1) functions both as a nonhistone nucleoprotein and an extracellular inflammatory cytokine. It is mainly located in the nucleus in the resting state and regulates key nuclear activities. After spinal cord injury, HMGB1 is expressed by neurons, microglia, and ependymal cells and released into the extracellular matrix and blood circulation, contributing to the pathophysiological process of spinal cord injury. HMGB1 can regulate microglia activation, exacerbate inflammation, and result in neuronal death. However, it has also been shown to benefit neuron survival, regeneration, and motor function recovery. This article reviews the timing of HMGB1 secretion and translocation, release mechanisms, and its role in spinal cord injury, and identifies challenges to be addressed.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Biology
Felix Buchner, Zeynep Dokuzluoglu, Tobias Grass, Natalia Rodriguez-Muela
Summary: Motor neuron diseases (MNDs) are a group of disorders that affect motor neurons and the muscular system. Despite decades of research, there is still limited understanding of the underlying molecular mechanisms and lack of effective therapies. Human 3D in vitro models, including spinal cord organoids, have revolutionized disease modeling and offer new perspectives for studying neurodevelopmental and neurodegenerative diseases.
Article
Biochemistry & Molecular Biology
Ning Wang, Hai Yu, Qian Song, Ping Mao, Kuo Li, Gang Bao
Summary: The study developed and characterized SM@SA-CS nanomicelles, showing potential therapeutic effects in oxidative stress in neural cells.
INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES
(2021)
Article
Oncology
Larisa Ryskalin, Rosangela Ferese, Gabriele Morucci, Francesca Biagioni, Carla L. Busceti, Fabrizio Michetti, Paola Lenzi, Alessandro Frati, Francesco Fornai
Summary: Accumulation of alpha-synuclein is a hallmark of neurodegenerative disorders and its clearance depends on autophagy activity. This study found that alpha-synuclein is overexpressed in glioblastoma multiforme cells and includes a proteinase K-resistant isoform.
Article
Biochemistry & Molecular Biology
Paola Lenzi, Francesca Biagioni, Carla L. Busceti, Gloria Lazzeri, Maico Polzella, Alessandro Frati, Michela Ferrucci, Francesco Fornai
Summary: Recent evidence suggests that methamphetamine (METH) causes mitochondrial alterations, leading to neurotoxicity. However, most studies have focused on mitochondrial activity rather than morphology. In this study, researchers used light and transmission electron microscopy to analyze dose-dependent mitochondrial structural changes during METH exposure. The findings indicate that METH-induced neurodegeneration is closely associated with specific mitochondrial damage, and a scoring system for mitochondrial integrity was established. Furthermore, the study revealed changes in mitochondrial proteins related to fission/mitophagy and mitochondrial dynamics. These results provide structural evidence that mitochondria are direct and indirect targets of METH-induced toxicity.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Biochemistry & Molecular Biology
Daniele Armocida, Carla Letizia Busceti, Francesca Biagioni, Francesco Fornai, Alessandro Frati
Summary: The complex carcinogenesis of glial tumors involves many genetic and epigenetic phenomena. Cellular prion protein (PrPC) and autophagy play important roles in cell death and tumorigenesis, and their interaction is involved in glioma carcinogenesis. Despite limited research on their combined activity, understanding their molecular mechanisms and pathways can provide valuable insights into the origin and treatment of glioma. This study explores the role of PrPC in autophagy processes and its interplay with glioma tumorigenesis, providing a basis for future research.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Cell Biology
Paola Lenzi, Carla L. L. Busceti, Gloria Lazzeri, Rosangela Ferese, Francesca Biagioni, Alessandra Salvetti, Elena Pompili, Valerio De Franchis, Stefano Puglisi-Allegra, Alessandro Frati, Michela Ferrucci, Francesco Fornai
Summary: Cells from glioblastoma multiforme (GBM) show increased levels of the mechanistic Target of Rapamycin (mTOR), which adversely affects malignancy and disease progression. The up-regulation of mTOR inhibits autophagy, which is important for the neurobiology of GBM. This study investigates the relationship between abnormal levels of cellular prion protein (PrPc), mitochondrial alterations, and mTOR inhibition in GBM cells.
Review
Biochemistry & Molecular Biology
Roberto Pinelli, Michela Ferrucci, Caterina Berti, Francesca Biagioni, Elena Scaffidi, Violet Vakunseth Bumah, Carla L. Busceti, Paola Lenzi, Gloria Lazzeri, Francesco Fornai
Summary: This article discusses the role of light in altering autophagy in both the outer retina and inner choroid. Autophagy is crucial for maintaining metabolic requirements and providing physiological activity for vision. The activation or inhibition of autophagy in the retinal pigment epithelium and outer segment of photoreceptors is dependent on light exposure. This also affects the choroid's involvement in providing blood flow and metabolic substrates. Dysfunction in autophagy can lead to degenerative conditions like age-related macular degeneration (AMD), highlighting the importance of understanding autophagy status in this area.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Giuseppe Pepe, Paola Lenzi, Luca Capocci, Federico Marracino, Ludovica Pizzati, Pamela Scarselli, Alba Di Pardo, Francesco Fornai, Vittorio Maglione
Summary: Huntington's disease is a neurodegenerative disorder caused by a mutation in huntingtin protein, and glycosphingolipid dysfunction may play a major role in its pathogenesis. In this study, the potential link between sphingolipid modulation and myelin structure was investigated. The glycosphingolipid modulator THI treatment preserved myelin thickness and structure, reduced pathologically giant axons, and restored the expression of myelin marker proteins. It also increased levels of GM1, which is associated with reduced toxicity of mutant huntingtin.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Medicine, General & Internal
Daniele Armocida, Alessandro Pesce, Mauro Palmieri, Fabio Cofano, Giuseppe Palmieri, Paola Cassoni, Carla Letizia Busceti, Francesca Biagioni, Diego Garbossa, Francesco Fornai, Antonio Santoro, Alessandro Frati
Summary: This study investigated the relationship between EGFR mutation and the disease course, prognosis, and diagnostic imaging of brain metastases (BMs) from non-small-cell lung cancer (NSCLC). The results showed a significant correlation between EGFR mutation and the size and edema volume of BMs, as well as the incidence of seizures.
JOURNAL OF CLINICAL MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Roberto Pinelli, Michela Ferrucci, Francesca Biagioni, Caterina Berti, Violet Vakunseth Bumah, Carla Letizia Busceti, Stefano Puglisi-Allegra, Gloria Lazzeri, Alessandro Frati, Francesco Fornai
Summary: The role of autophagy in age-related macular degeneration (AMD) is to clear reactive oxidative species and dysfunctional mitochondria. Impaired autophagy leads to the accumulation of reactive oxygen species, causing retinal degeneration. Autophagy in retinal pigment epithelium (RPE) is induced by stimuli like light and phytochemicals, and their synergistic effects enhance retinal structure and visual acuity. Light-dependent beneficial antioxidant effects can be achieved by combining light pulses with phytochemicals in AMD.
Review
Medicine, General & Internal
Lorenzo Pescatori, Samanta Taurone, Antonello Ciccarelli, Mauro Palmieri, Alessandra Serraino, Marco Artico, Francesco Fornai, Yaroslava Longhitano, Christian Zanza, Manfredi Tesauro, Gabriele Savioli, Selenia Miglietta, Pasqualino Ciappetta
Summary: A systematic literature review was conducted to describe the anatomical region located at the junction of the sphenoid and the basal portion of the temporal bone. The study focused on the relationships between the anterior margin of the free edge of the tentorium and the components of the sphenoid and petrous bone.
