Characterization of the expression of dystrophins and dystrophin-associated proteins during embryonic neural stem/progenitor cell differentiation

Romo-Yáñez, José; Rodríguez‐Martínez, Griselda; Aragón, Jorge; Siqueiros‐Márquez, Lourdes; Herrera‐Salazar, Alma; Velasco, Iván; Montañéz, Cecilia

doi:10.1016/j.neulet.2020.135247

Cited by 17 publications

(18 citation statements)

References 30 publications

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“…The various dystrophins are encoded by the 79 exon-spanning DMD gene, whereby seven different promoters drive the tissue-specific expression of the full-length isoforms Dp427-B in brain, Dp427-M in muscle and Dp427-P in Purkinje cells [4], as well as the shorter isoforms Dp260-R in retina [79], Dp140-B/K in brain and kidney [80], Dp116-S in Schwann cells [81] and Dp71-G in the brain [82] and a variety of other tissues including the spleen [83]. The promoter for Dp71 also produces the shortest known dystrophin isoform named Dp45, which is located in the central nervous system [84]. Of note, the central nervous system displays one of the greatest varieties of dystrophin isoforms, which are involved in synaptic modulation, neuronal excitability and signal integration.…”

Section: Muscle Dystrophin Dp427-m and Its Associated Glycoprotein Complexmentioning

confidence: 99%

The Dystrophin Node as Integrator of Cytoskeletal Organization, Lateral Force Transmission, Fiber Stability and Cellular Signaling in Skeletal Muscle

et al. 2021

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The systematic bioanalytical characterization of the protein product of the DMD gene, which is defective in the pediatric disorder Duchenne muscular dystrophy, led to the discovery of the membrane cytoskeletal protein dystrophin. Its full-length muscle isoform Dp427-M is tightly linked to a sarcolemma-associated complex consisting of dystroglycans, sarcoglyans, sarcospan, dystrobrevins and syntrophins. Besides these core members of the dystrophin–glycoprotein complex, the wider dystrophin-associated network includes key proteins belonging to the intracellular cytoskeleton and microtubular assembly, the basal lamina and extracellular matrix, various plasma membrane proteins and cytosolic components. Here, we review the central role of the dystrophin complex as a master node in muscle fibers that integrates cytoskeletal organization and cellular signaling at the muscle periphery, as well as providing sarcolemmal stabilization and contractile force transmission to the extracellular region. The combination of optimized tissue extraction, subcellular fractionation, advanced protein co-purification strategies, immunoprecipitation, liquid chromatography and two-dimensional gel electrophoresis with modern mass spectrometry-based proteomics has confirmed the composition of the core dystrophin complex at the sarcolemma membrane. Importantly, these biochemical and mass spectrometric surveys have identified additional members of the wider dystrophin network including biglycan, cavin, synemin, desmoglein, tubulin, plakoglobin, cytokeratin and a variety of signaling proteins and ion channels.

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Section: Muscle Dystrophin Dp427-m and Its Associated Glycoprotein Complexmentioning

confidence: 99%

The Dystrophin Node as Integrator of Cytoskeletal Organization, Lateral Force Transmission, Fiber Stability and Cellular Signaling in Skeletal Muscle

et al. 2021

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“…These antibodies were used in this data article, while the following antibodies: α1CT-FP, a pAb for α-dystrobrevin kindly provided by Blake et al. [8] , mAb anti-dystrophins (Dys2) (NCL-DYS2, Novocastra), mAb anti-Sox2 (MAB2018, R&D), mAb anti-GFAP (MMS-588S, Covance), and mAb anti-microtubule-associated protein 2 (MAP2) (AB11267, Covance) were used in the principal article [4] .…”

Section: Methods Detailsmentioning

confidence: 99%

“…The detection of the immunoreactive bands was carried out using the Western Lightning ECL-Plus system (Perkin-Elmer, Inc.). The antibodies H4 (1:5000), JAF (1:5000), and α1CT-FP (1:1000) were used in the principal article [4] .…”

Section: Methods Detailsmentioning

confidence: 99%

“…The images were analyzed using the Leica Application Suite AF software (Leica). The mAb anti-Sox2 (1:200), mAb anti-MAP2 (1:500), pAb anti-GFAP (1:2000), and mAb anti-GFAP (1:500) were used in the principal article [4] .…”

Section: Methods Detailsmentioning

confidence: 99%

“…2 ). Additionally, expression of dystrophins and dystrophin-associated proteins complex (DAPC) in proliferating and differentiating NSPC was characterized [4] .

Fig.…”

Section: Introductionmentioning

confidence: 99%

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Embryonic neural stem/progenitor cells as model to characterize dystrophin and dystrophin-associated proteins expression during neuronal or astrocytic differentiation

et al. 2021

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Neural stem/progenitor cells (NSPC) are multipotent cells that renew themselves and could differentiate into neurons and macro glia (astrocytes and oligodendrocytes) of the nervous system during embryonic development. Duchenne muscular dystrophy is a severe type of muscular dystrophy caused by mutations in the dmd gene, and one-third of patients cursed with neuro-cognitive impairments. In this data article, we take advantage of the differentiation capacity of NSPC as a model to increase our knowledge in the neuronal and/or astrocytic differentiation and to evaluate the expression of dystrophins and dystrophin-associated proteins. We showed the characterization of undifferentiated and neuron and/or astrocyte differentiated NSPC. In addition, we evaluated the expression and subcellular localization of dystrophins and β-dystroglycan in undifferentiated NSPC and differentiated to neurons and astrocytes. Primary culture of NSPC was characterized by the expression of multipotent markers nestin and Sox2. Neuronal or astrocytic differentiation of NSPC was performed by basic fibroblast growth factor (FGF2) withdrawal, histamine or ciliary neurotrophic factor (CNTF) treatment, and expression of βIII-tubulin or glial fibrillary acidic protein (GFAP) as differentiation markers for neurons or astrocytes was evaluated. This study will contribute to the understanding of dystrophins and dystrophin-associated proteins expression and function during neuronal or astrocytic differentiation of NSPC

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Muscular Dystrophy: Underlying Cellular and Molecular Mechanisms and Various Nanotherapeutic Approaches for Muscular Dystrophy

Syed,

Khan,

Afnan

et al. 2024

Mechanism and Genetic Susceptibility of Neurological Disorders

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Characterization of the expression of dystrophins and dystrophin-associated proteins during embryonic neural stem/progenitor cell differentiation

Cited by 17 publications

References 30 publications

The Dystrophin Node as Integrator of Cytoskeletal Organization, Lateral Force Transmission, Fiber Stability and Cellular Signaling in Skeletal Muscle

The Dystrophin Node as Integrator of Cytoskeletal Organization, Lateral Force Transmission, Fiber Stability and Cellular Signaling in Skeletal Muscle

Embryonic neural stem/progenitor cells as model to characterize dystrophin and dystrophin-associated proteins expression during neuronal or astrocytic differentiation

Muscular Dystrophy: Underlying Cellular and Molecular Mechanisms and Various Nanotherapeutic Approaches for Muscular Dystrophy

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