In Vitro Models of Amyotrophic Lateral Sclerosis

Zhou, Lijun; Chen, Wenzhi; Jiang, Shishi; Xu, Renshi

doi:10.1007/s10571-023-01423-8

Cited by 9 publications

(4 citation statements)

References 126 publications

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“…The implementation of such engineered models holds great promise in studying the pathophysiological mechanisms of diseases associated with the NMJ, such as amyotrophic lateral sclerosis (ALS). The use of iPSCs to generate neural and muscular tissues facilitates the generation of patient-derived NMJ models to elucidate the pathophysiological hallmarks of NMJ degeneration that are specific to each patient [304]. Recent developments in the field have proven fruitful with the creation of compartmentalized models that recreate both the neuronal and the muscular components of the NMJ.…”

Section: Modeling Aging-related Neuromuscular Junction Degenerationmentioning

confidence: 99%

“…Recent developments in the field have proven fruitful with the creation of compartmentalized models that recreate both the neuronal and the muscular components of the NMJ. When neurons and muscle cells are cultured in proximity, the axons of neurons extend into the myofibers and form the NMJ [304]. A representative example of such a system is the model developed by Osaki et al, which consists of a compartmentalized platform comprised of iPSC-derived motor neurons from patients in the form of spheroids and skeletal muscle bundles.…”

Section: Modeling Aging-related Neuromuscular Junction Degenerationmentioning

confidence: 99%

“…As previously mentioned, microfluidic platforms have distinctive advantages over other systems with regard to mechanical, biochemical, and electrical stimulation [309,310]. Furthermore, the observation of the nerve impulse transmission is facilitated by the transparency of the materials used in the manufacture of these organon-a-chip platforms [304]. This feature enables the integration of sensors to monitor the cellular response to biophysical stimuli and drugs [311].…”

Section: Modeling Aging-related Neuromuscular Junction Degenerationmentioning

confidence: 99%

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Leveraging Biomaterial Platforms to Study Aging-Related Neural and Muscular Degeneration

Hidalgo-Alvarez,

Madl

2024

Biomolecules

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Aging is a complex multifactorial process that results in tissue function impairment across the whole organism. One of the common consequences of this process is the loss of muscle mass and the associated decline in muscle function, known as sarcopenia. Aging also presents with an increased risk of developing other pathological conditions such as neurodegeneration. Muscular and neuronal degeneration cause mobility issues and cognitive impairment, hence having a major impact on the quality of life of the older population. The development of novel therapies that can ameliorate the effects of aging is currently hindered by our limited knowledge of the underlying mechanisms and the use of models that fail to recapitulate the structure and composition of the cell microenvironment. The emergence of bioengineering techniques based on the use of biomimetic materials and biofabrication methods has opened the possibility of generating 3D models of muscular and nervous tissues that better mimic the native extracellular matrix. These platforms are particularly advantageous for drug testing and mechanistic studies. In this review, we discuss the developments made in the creation of 3D models of aging-related neuronal and muscular degeneration and we provide a perspective on the future directions for the field.

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Section: Modeling Aging-related Neuromuscular Junction Degenerationmentioning

confidence: 99%

Section: Modeling Aging-related Neuromuscular Junction Degenerationmentioning

confidence: 99%

Section: Modeling Aging-related Neuromuscular Junction Degenerationmentioning

confidence: 99%

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Leveraging Biomaterial Platforms to Study Aging-Related Neural and Muscular Degeneration

Hidalgo-Alvarez,

Madl

2024

Biomolecules

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“…Mutant- SOD1 mouse models have been used extensively to investigate ALS pathology, but drawbacks associated with animal models led to difficulties in translation to human pathology. To bridge the gap, in vitro models have been developed as recently reviewed ( Zhou et al, 2023 ). Human induced pluripotent stem cells (hiPSC) can be exploited for a deeper understanding of developmental biology, disease modeling, and testing of drugs or therapeutic efficiency ( Okano et al, 2023 ) and are therefore valuable tools for addressing ALS pathogenesis.…”

Section: Introductionmentioning

confidence: 99%

Aberrant evoked calcium signaling and nAChR cluster morphology in a SOD1 D90A hiPSC-derived neuromuscular model

Couturier,

Hörner,

Nürnberg

et al. 2024

Front. Cell Dev. Biol.

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Familial amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disorder that is due to mutations in one of several target genes, including SOD1. So far, clinical records, rodent studies, and in vitro models have yielded arguments for either a primary motor neuron disease, or a pleiotropic pathogenesis of ALS. While mouse models lack the human origin, in vitro models using human induced pluripotent stem cells (hiPSC) have been recently developed for addressing ALS pathogenesis. In spite of improvements regarding the generation of muscle cells from hiPSC, the degree of maturation of muscle cells resulting from these protocols has remained limited. To fill these shortcomings, we here present a new protocol for an enhanced myotube differentiation from hiPSC with the option of further maturation upon coculture with hiPSC-derived motor neurons. The described model is the first to yield a combination of key myogenic maturation features that are consistent sarcomeric organization in association with complex nAChR clusters in myotubes derived from control hiPSC. In this model, myotubes derived from hiPSC carrying the SOD1 D90A mutation had reduced expression of myogenic markers, lack of sarcomeres, morphologically different nAChR clusters, and an altered nAChR-dependent Ca2+ response compared to control myotubes. Notably, trophic support provided by control hiPSC-derived motor neurons reduced nAChR cluster differences between control and SOD1 D90A myotubes. In summary, a novel hiPSC-derived neuromuscular model yields evidence for both muscle-intrinsic and nerve-dependent aspects of neuromuscular dysfunction in SOD1-based ALS.

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Overview of nomenclature and diagnosis of amyotrophic lateral sclerosis

2024

Annals of Medicine

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The nomenclature of amyotrophic lateral sclerosis (ALS) currently is blurred, indistinct and no accurate and haven’t been properly updated since the first description, which is far from being suitable for the current implementation of clinical practise and scientific research of ALS, and urgently need an solution. Furthermore, the current diagnostic criteria need also further been improved, because the current clinical diagnosis of ALS majorly depends on the clinical manifestations yet. Up to now, no any objective clinical auxiliary examination can be helpful to diagnose ALS besides the electromyogram identifying the lower motor neuron damage, which isn’t conducive to early diagnosis and prolongs the time of ALS confirmed diagnosis. In this mini review, we discussed the current doubt about the nomenclature and diagnostic criteria of ALS, and prospected in order to further improve and normalize the nomenclature and diagnosis of ALS.

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In Vitro Models of Amyotrophic Lateral Sclerosis

Cited by 9 publications

References 126 publications

Leveraging Biomaterial Platforms to Study Aging-Related Neural and Muscular Degeneration

Leveraging Biomaterial Platforms to Study Aging-Related Neural and Muscular Degeneration

Aberrant evoked calcium signaling and nAChR cluster morphology in a SOD1 D90A hiPSC-derived neuromuscular model

Overview of nomenclature and diagnosis of amyotrophic lateral sclerosis

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