XB-ART-60369
Cell Mol Neurobiol
2023 Nov 01;438:3783-3799. doi: 10.1007/s10571-023-01423-8.
Show Gene links
Show Anatomy links
In Vitro Models of Amyotrophic Lateral Sclerosis.
Zhou L
,
Chen W
,
Jiang S
,
Xu R
.
???displayArticle.abstract???
Amyotrophic Lateral Sclerosis (ALS) is one of the commonest neurodegenerative diseases of adult-onset, which is characterized by the progressive death of motor neurons in the cerebral cortex, brain stem and spinal cord. The dysfunction and death of motor neurons lead to the progressive muscle weakness, atrophy, fasciculations, spasticity and ultimately the whole paralysis of body. Despite the identification of several genetic mutations associated with the pathogenesis of ALS, including mutations in chromosome 9 open reading frame 72 leading to the abnormal expansion of GGGGCC repeat sequence, TAR DNA-binding protein 43, fused in sarcoma/translocated in liposarcoma, copper/zinc superoxide dismutase 1 (SOD1) and TANK-binding kinase 1, the exact mechanisms underlying the specific degeneration of motor neurons that causes ALS remain incompletely understood. At present, since the transgenic model expressed SOD1 mutants was established, multiple in vitro models of ALS have been developed for studying the pathology, pathophysiology and pathogenesis of ALS as well as searching the effective neurotherapeutics. This review reviewed the details of present established in vitro models used in studying the pathology, pathophysiology and pathogenesis of ALS. Meanwhile, we also discussed the advantages, disadvantages, cost and availability of each models.
???displayArticle.pubmedLink??? 37870685
???displayArticle.link??? Cell Mol Neurobiol
???displayArticle.grants??? [+]
30560042 National Natural Science Foundation of China, 81160161 National Natural Science Foundation of China, 81360198 National Natural Science Foundation of China, 82160255 National Natural Science Foundation of China, GJJ13198 Education Department of Jiangxi Province, GJJ170021 Education Department of Jiangxi Province, 20192BAB205043 Jiangxi Provincial Department of Science and Technology, 20181019 Health and Family Planning Commission of Jiangxi Province, 202210002 Health and Family Planning Commission of Jiangxi Province
Genes referenced: sod1
References [+] :
Afshar Bakooshli,
A 3D culture model of innervated human skeletal muscle enables studies of the adult neuromuscular junction.
2019, Pubmed
Afshar Bakooshli, A 3D culture model of innervated human skeletal muscle enables studies of the adult neuromuscular junction. 2019, Pubmed
AlaylioĞlu, A Bridge Between in vitro and in vivo Studies in Neuroscience: Organotypic Brain Slice Cultures. 2020, Pubmed
Allen, Improving physiological relevance of cell culture: the possibilities, considerations, and future directions of the ex vivo coculture model. 2023, Pubmed
Amorós, Motor neuron-derived induced pluripotent stem cells as a drug screening platform for amyotrophic lateral sclerosis. 2022, Pubmed
Balendra, C9orf72-mediated ALS and FTD: multiple pathways to disease. 2018, Pubmed
Baloh, Transplantation of human neural progenitor cells secreting GDNF into the spinal cord of patients with ALS: a phase 1/2a trial. 2022, Pubmed
Barber, An in vitro screening cascade to identify neuroprotective antioxidants in ALS. 2009, Pubmed
Bauchet, Isolation and culture of precursor cells from the adult human spinal cord. 2013, Pubmed
Boehmer, Post-translational regulation of EAAT2 function by co-expressed ubiquitin ligase Nedd4-2 is impacted by SGK kinases. 2006, Pubmed , Xenbase
Boido, Organotypic spinal cord cultures: An <em>in vitro</em> 3D model to preliminary screen treatments for spinal muscular atrophy. 2021, Pubmed
Bonaventura, PACAP and PAC1R are differentially expressed in motor cortex of amyotrophic lateral sclerosis patients and support survival of iPSC-derived motor neurons. 2018, Pubmed
Butti, Reduced C9orf72 function leads to defective synaptic vesicle release and neuromuscular dysfunction in zebrafish. 2021, Pubmed
Bär, Motor neuron disease in vitro: the use of cultured motor neurons to study amyotrophic lateral sclerosis. 2000, Pubmed
Cacabelos, Interplay between TDP-43 and docosahexaenoic acid-related processes in amyotrophic lateral sclerosis. 2016, Pubmed
Calderó, Lithium prevents excitotoxic cell death of motoneurons in organotypic slice cultures of spinal cord. 2010, Pubmed
Cappella, Gene Therapy for ALS-A Perspective. 2019, Pubmed
Cashman, Neuroblastoma x spinal cord (NSC) hybrid cell lines resemble developing motor neurons. 1992, Pubmed
Chia, Novel genes associated with amyotrophic lateral sclerosis: diagnostic and clinical implications. 2018, Pubmed
Cifra, Riluzole is a potent drug to protect neonatal rat hypoglossal motoneurons in vitro from excitotoxicity due to glutamate uptake block. 2011, Pubmed
Cifra, Respiratory motoneurons and pathological conditions: lessons from hypoglossal motoneurons challenged by excitotoxic or oxidative stress. 2011, Pubmed
Cullen, In vitro neural injury model for optimization of tissue-engineered constructs. 2007, Pubmed
De Paola, Morphological features and responses to AMPA receptor-mediated excitotoxicity of mouse motor neurons: comparison in purified, mixed anterior horn or motor neuron/glia cocultures. 2008, Pubmed
Delfs, Ventral and dorsal horn acetylcholinesterase neurons are maintained in organotypic cultures of postnatal rat spinal cord explants. 1989, Pubmed
Di Giorgio, Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model. 2007, Pubmed
Dimos, Induced pluripotent stem cells generated from patients with ALS can be differentiated into motor neurons. 2008, Pubmed
Du, Circadian regulation of developmental synaptogenesis via the hypocretinergic system. 2023, Pubmed
Durham, Evaluation of the spinal cord neuron X neuroblastoma hybrid cell line NSC-34 as a model for neurotoxicity testing. 1993, Pubmed
Elkhenany, A Hyaluronic Acid Demilune Scaffold and Polypyrrole-Coated Fibers Carrying Embedded Human Neural Precursor Cells and Curcumin for Surface Capping of Spinal Cord Injuries. 2021, Pubmed
Faravelli, Motor neuron derivation from human embryonic and induced pluripotent stem cells: experimental approaches and clinical perspectives. 2014, Pubmed
Foran, Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2. 2011, Pubmed
Gingras, In vitro study of axonal migration and myelination of motor neurons in a three-dimensional tissue-engineered model. 2008, Pubmed
Gingras, Differentiation of human adult skin-derived neuronal precursors into mature neurons. 2007, Pubmed
Gingras, Optimized protocols for isolation of primary motor neurons, astrocytes and microglia from embryonic mouse spinal cord. 2007, Pubmed
Gois, IN VITRO AND IN VIVO MODELS OF AMYOTROPHIC LATERAL SCLEROSIS: AN UPDATED OVERVIEW. 2020, Pubmed
Gore, The zebrafish: A fintastic model for hematopoietic development and disease. 2018, Pubmed
Haidet-Phillips, Astrocytes from familial and sporadic ALS patients are toxic to motor neurons. 2011, Pubmed
Hester, Rapid and efficient generation of functional motor neurons from human pluripotent stem cells using gene delivered transcription factor codes. 2011, Pubmed
Hoffman, Chronic neocortical epileptogenesis in vitro. 1994, Pubmed
Hu, Differentiation of spinal motor neurons from pluripotent human stem cells. 2009, Pubmed
Humpel, Organotypic Brain Slices of ADULT Transgenic Mice: A Tool to Study Alzheimer's Disease. 2019, Pubmed
Hunter, An LRE (leucine-arginine-glutamate)-dependent mechanism for adhesion of neurons to S-laminin. 1991, Pubmed
Hwang, Regulation of RNA localization during oocyte maturation by dynamic RNA-ER association and remodeling of the ER. 2022, Pubmed , Xenbase
Johns, Exploring Motor Neuron Diseases Using iPSC Platforms. 2022, Pubmed
Kalueff, Zebrafish as an emerging model for studying complex brain disorders. 2014, Pubmed
Kasper, Mimicking exercise in three-dimensional bioengineered skeletal muscle to investigate cellular and molecular mechanisms of physiological adaptation. 2018, Pubmed
Kim, ALS Genetics: Gains, Losses, and Implications for Future Therapies. 2020, Pubmed
Konopka, Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations. 2020, Pubmed
Kosuge, Characterization of chronic glutamate-mediated motor neuron toxicity in organotypic spinal cord culture prepared from ALS model mice. 2009, Pubmed
Kulshreshtha, Vascular endothelial growth factor attenuates neurodegenerative changes in the NSC-34 motor neuron cell line induced by cerebrospinal fluid of sporadic amyotrophic lateral sclerosis patients. 2011, Pubmed
Kwiatkowski, Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. 2009, Pubmed
Laflamme, Implementation of an antibody characterization procedure and application to the major ALS/FTD disease gene C9ORF72. 2019, Pubmed
Lee, Human in vitro systems for examining synaptic function and plasticity in the brain. 2020, Pubmed
Li, Neuromuscular Junction Formation, Aging, and Disorders. 2018, Pubmed
Linares, SYF2 suppression mitigates neurodegeneration in models of diverse forms of ALS. 2023, Pubmed
Liu, Protein S confers neuronal protection during ischemic/hypoxic injury in mice. 2003, Pubmed
Liu, BL-918, a small-molecule activator of ULK1, induces cytoprotective autophagy for amyotrophic lateral sclerosis therapy. 2023, Pubmed
Lu, Lipin 1 deficiency causes adult-onset myasthenia with motor neuron dysfunction in humans and neuromuscular junction defects in zebrafish. 2021, Pubmed
Lunn, Vascular endothelial growth factor prevents G93A-SOD1-induced motor neuron degeneration. 2009, Pubmed
Mackenzie, TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. 2010, Pubmed
Malik, A review of the methods for human iPSC derivation. 2013, Pubmed
Mazzone, Electrochemical detection of endogenous glutamate release from rat spinal cord organotypic slices as a real-time method to monitor excitotoxicity. 2011, Pubmed
Mckee, The neuropathology of traumatic brain injury. 2015, Pubmed
Mitne-Neto, Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients. 2011, Pubmed
Morrison, Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis. NULL, Pubmed
Moyle, Innovation in culture systems to study muscle complexity. 2022, Pubmed
Nicoll, Synaptic memory and CaMKII. 2023, Pubmed
Okano, Transplantation of neural progenitor cells into the human CNS. 2022, Pubmed
Okano, iPSC-based disease modeling and drug discovery in cardinal neurodegenerative disorders. 2022, Pubmed
Oliveira, Swimming against ALS: How to model disease in zebrafish for pathophysiological and behavioral studies. 2023, Pubmed
Pakan, A method to investigate radial glia cell behavior using two-photon time-lapse microscopy in an ex vivo model of spinal cord development. 2014, Pubmed
Penney, Modeling Alzheimer's disease with iPSC-derived brain cells. 2020, Pubmed
Pensalfini, Intracellular Injection of Brain Extracts from Alzheimer's Disease Patients Triggers Unregulated Ca2+ Release from Intracellular Stores That Hinders Cellular Bioenergetics. 2022, Pubmed , Xenbase
Pereira, Microglial depletion exacerbates motor impairment and dopaminergic neuron loss in a 6-OHDA model of Parkinson's disease. 2023, Pubmed
Prpar Mihevc, Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle. 2017, Pubmed
Qi, Human Brain Slice Culture: A Useful Tool to Study Brain Disorders and Potential Therapeutic Compounds. 2019, Pubmed
Rizzuti, Insights into the identification of a molecular signature for amyotrophic lateral sclerosis exploiting integrated microRNA profiling of iPSC-derived motor neurons and exosomes. 2022, Pubmed
Rosen, Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. 1993, Pubmed
Roudsari, GMP-Based Isolation of Full-Term Human Placenta-Derived NK Cells for CAR-NK Cell Therapy in Malignant Melanoma. 2023, Pubmed
Salomon-Zimri, Combination of ciprofloxacin/celecoxib as a novel therapeutic strategy for ALS. 2023, Pubmed
Salvador, An In Vitro Model of Traumatic Brain Injury. 2018, Pubmed
Sanelli, Calcium mediated excitotoxicity in neurofilament aggregate-bearing neurons in vitro is NMDA receptor dependant. 2007, Pubmed
Scarian, Patients' Stem Cells Differentiation in a 3D Environment as a Promising Experimental Tool for the Study of Amyotrophic Lateral Sclerosis. 2022, Pubmed
Schmid, Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth. 2013, Pubmed
Schnaar, Separation of cell types from embryonic chicken and rat spinal cord: characterization of motoneuron-enriched fractions. 1981, Pubmed
Sepehrimanesh, Generation and optimization of highly pure motor neurons from human induced pluripotent stem cells via lentiviral delivery of transcription factors. 2020, Pubmed
Shi, Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export. 2017, Pubmed , Xenbase
Shoemaker, Untangling the cortex: Advances in understanding specification and differentiation of corticospinal motor neurons. 2010, Pubmed
Stabenfeldt, Biomimetic microenvironment modulates neural stem cell survival, migration, and differentiation. 2010, Pubmed
Syyam, Adenovirus vector system: construction, history and therapeutic applications. 2022, Pubmed
Szebényi, Human ALS/FTD brain organoid slice cultures display distinct early astrocyte and targetable neuronal pathology. 2021, Pubmed
Tabata, Chronic exposure to dietary sterol glucosides is neurotoxic to motor neurons and induces an ALS-PDC phenotype. 2008, Pubmed
Taga, Establishment of an Electrophysiological Platform for Modeling ALS with Regionally-Specific Human Pluripotent Stem Cell-Derived Astrocytes and Neurons. 2021, Pubmed
Takahashi, Induction of pluripotent stem cells from fibroblast cultures. 2007, Pubmed
Takahashi, Induction of pluripotent stem cells from mouse embryonic and adult fibroblast cultures by defined factors. 2006, Pubmed
Taylor, Prematurely terminated intron-retaining mRNAs invade axons in SFPQ null-driven neurodegeneration and are a hallmark of ALS. 2022, Pubmed
Texidó, Sera from amyotrophic lateral sclerosis patients induce the non-canonical activation of NMDA receptors "in vitro". 2011, Pubmed , Xenbase
Tian, CRISPR Interference-Based Platform for Multimodal Genetic Screens in Human iPSC-Derived Neurons. 2019, Pubmed
Toma, Isolation of multipotent adult stem cells from the dermis of mammalian skin. 2001, Pubmed
Tovar-Y-Romo, Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis. 2009, Pubmed
Tradewell, Calcium dysregulation, mitochondrial pathology and protein aggregation in a culture model of amyotrophic lateral sclerosis: mechanistic relationship and differential sensitivity to intervention. 2011, Pubmed
Tradewell, Calpastatin reduces toxicity of SOD1G93A in a culture model of amyotrophic lateral sclerosis. 2010, Pubmed
Trotti, Amyotrophic lateral sclerosis-linked glutamate transporter mutant has impaired glutamate clearance capacity. 2001, Pubmed , Xenbase
Trotti, SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter. 1999, Pubmed , Xenbase
Tscherter, Spatiotemporal characterization of rhythmic activity in rat spinal cord slice cultures. 2001, Pubmed
Ucar, Collagen hydrogels loaded with fibroblast growth factor-2 as a bridge to repair brain vessels in organotypic brain slices. 2020, Pubmed
Vijayalakshmi, Evidence of endoplasmic reticular stress in the spinal motor neurons exposed to CSF from sporadic amyotrophic lateral sclerosis patients. 2011, Pubmed
Vijayalakshmi, Cerebrospinal fluid from sporadic amyotrophic lateral sclerosis patients induces degeneration of a cultured motor neuron cell line. 2009, Pubmed
Wakamori, Quantification of the effect of site-specific histone acetylation on chromatin transcription rate. 2020, Pubmed , Xenbase
Wan, Emerging Roles of Microfluidics in Brain Research: From Cerebral Fluids Manipulation to Brain-on-a-Chip and Neuroelectronic Devices Engineering. 2022, Pubmed
Wang, Viral vector-mediated gene therapy for opioid use disorders. 2021, Pubmed
Wichterle, Directed differentiation of embryonic stem cells into motor neurons. 2002, Pubmed
Wong, Systematic, comprehensive, evidence-based approach to identify neuroprotective interventions for motor neuron disease: using systematic reviews to inform expert consensus. 2023, Pubmed
Wood, Molecular Mechanisms Underlying TDP-43 Pathology in Cellular and Animal Models of ALS and FTLD. 2021, Pubmed
Workman, Large-scale differentiation of iPSC-derived motor neurons from ALS and control subjects. 2023, Pubmed
Yang, The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism. 2010, Pubmed
Young, Glutamate receptor expression and chronic glutamate toxicity in rat motor cortex. 2007, Pubmed
Yu, TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS. 2020, Pubmed
Zeng, Using Xenopus oocytes in neurological disease drug discovery. 2020, Pubmed , Xenbase
Zhou, Honokiol alleviated neurodegeneration by reducing oxidative stress and improving mitochondrial function in mutant SOD1 cellular and mouse models of amyotrophic lateral sclerosis. 2023, Pubmed
de Jongh, Neuromuscular junction-on-a-chip: ALS disease modeling and read-out development in microfluidic devices. 2021, Pubmed