Advanced Cellular Models for Rare Disease Study: Exploring Neural, Muscle and Skeletal Organoids

Bombieri, Cristina; Corsi, Andrea; Trabetti, Elisabetta; Ruggiero, Alessandra; Marchetto, Giulia; Vattemi, Gaetano; Valenti, Maria Teresa; Zipeto, Donato; Romanelli, Maria Grazia

doi:10.3390/ijms25021014

Cited by 7 publications

(2 citation statements)

References 179 publications

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“…Bone homeostasis, the balance between bone formation and resorption, is mainly regulated by osteoblasts and osteoclasts [ 1 ]. Osteoblasts originate from bone marrow mesenchymal stem cells (BMSCs) and are bone-forming cells while osteoclasts derive from monocyte/macrophage lineage and are bone-resorption cells [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Exploring the Role of Circular RNA in Bone Biology: A Comprehensive Review

Valenti,

Zerlotin,

Cominacini

et al. 2024

Cells

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Circular RNAs (circRNAs) have emerged as pivotal regulators of gene expression with diverse roles in various biological processes. In recent years, research into circRNAs’ involvement in bone biology has gained significant attention, unveiling their potential as novel regulators and biomarkers in bone-related disorders and diseases. CircRNAs, characterized by their closed-loop structure, exhibit stability and resistance to degradation, underscoring their functional significance. In bone tissue, circRNAs are involved in critical processes such as osteogenic differentiation, osteoclastogenesis, and bone remodeling through intricate molecular mechanisms including microRNA regulation. Dysregulated circRNAs are associated with various bone disorders, suggesting their potential as diagnostic and prognostic biomarkers. The therapeutic targeting of these circRNAs holds promise for addressing bone-related conditions, offering new perspectives for precision medicine. Thus, circRNAs constitute integral components of bone regulatory networks, impacting both physiological bone homeostasis and pathological conditions. This review provides a comprehensive overview of circRNAs in bone biology, emphasizing their regulatory mechanisms, functional implications, and therapeutic potential.

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Section: Introductionmentioning

confidence: 99%

Exploring the Role of Circular RNA in Bone Biology: A Comprehensive Review

Valenti,

Zerlotin,

Cominacini

et al. 2024

Cells

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“…However, myotubes showed a limited differentiation level and the occurrence of nAChR plaques was rare ( Badu-Mensah et al, 2020 ). Other options used co-differentiation of muscle cells and motor neurons in a dish ( Mazaleyrat et al, 2020 ; Urzi et al, 2023 ) or organoid generation ( Faustino Martins et al, 2020 ; Bombieri et al, 2024 ).…”

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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Organoids of Musculoskeletal System for Disease Modeling, Drug Screening, and Regeneration

Chen,

Liu,

et al. 2024

Adv Healthcare Materials

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Musculoskeletal diseases have emerged as the leading cause of disability worldwide, with their prevalence increasing annually. In light of this escalating health challenge, organoids, an emerging technology in tissue engineering, offer promising solutions for disease modeling, drug screening, regeneration, and repair processes. The successful development of musculoskeletal organoids represents a significant breakthrough, providing a novel platform for studying musculoskeletal diseases and facilitating the discovery of new treatments. Moreover, organoids serve as valuable complements to traditional 2D culture methods and animal models, offering rich insights into musculoskeletal biology. This review provides an overview of organoid technology, outlining the construction processes of various musculoskeletal organoids and highlighting their similarities and differences. Furthermore, the challenges associated with organoid technology in musculoskeletal systems are discussed and insights into future perspectives are offered.

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Advanced Cellular Models for Rare Disease Study: Exploring Neural, Muscle and Skeletal Organoids

Cited by 7 publications

References 179 publications

Exploring the Role of Circular RNA in Bone Biology: A Comprehensive Review

Exploring the Role of Circular RNA in Bone Biology: A Comprehensive Review

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

Organoids of Musculoskeletal System for Disease Modeling, Drug Screening, and Regeneration

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