Ion channels in a skeletal muscle cell line from a Duchenne muscular dystrophy patient

Caviedes, Raúl; Caviedes, Pablo; Liberona, José Luis; Jaimovich, Enrique

doi:10.1002/mus.880170909

Cited by 14 publications

(12 citation statements)

References 27 publications

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“…6 Unlike other cell lines established by the same procedure, 7 RCDMD cells were highly refractory to transformation, and the resulting cell line grew slowly, with a doubling time of approximately 48 h. Some characteristics of the cell line include the presence of ion currents and receptors for the dihydropyridine PN200-110 and for ␣-bungarotoxin. 6 Unlike the human counterpart, the murine cell line described here has a tendency to form large myotubes with distinct myofibrillar organization upon differentiation; both dystrophic cell lines lack positive staining with antidystrophin antibodies.…”

Section: Discussionmentioning

confidence: 96%

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Differences in both inositol 1,4,5-trisphosphate mass and inositol 1,4,5-trisphosphate receptors between normal and dystrophic skeletal muscle cell lines

Liberona¹,

Powell²,

Shenoi³

et al. 1998

Muscle Nerve

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Human normal (RCMH) and Duchenne muscular dystrophy (RCDMD) cell lines, as well as newly developed normal and dystrophic murine cell lines, were used for the study of both changes in inositol 1,4,5‐trisphosphate (IP3) mass and IP3 binding to receptors. Basal levels of IP3 were increased two‐ to threefold in dystrophic human and murine cell lines compared to normal cell lines. Potassium depolarization induced a time‐dependent IP3 rise in normal human cells and cells of the myogenic mouse cell line (129CB3), which returned to their basal levels after 60 s. However, in the human dystrophic cell line (RCDMD), IP3 levels remained high up to 200 s after potassium depolarization. Expression of IP3 receptors was studied measuring specific binding of 3H‐IP3 in the murine cell lines (normal 129CB3 and dystrophic mdx XLT 4‐2). All the cell lines bind 3H‐IP3 with relatively high affinity (Kd: between 40 and 100 nmol/L). IP3 receptors are concentrated in the nuclear fraction, and their density is significantly higher in dystrophic cells compared to normal. These findings together with high basal levels of IP3 mass suggest a possible role for this system in the deficiency of intracellular calcium regulation in Duchenne muscular dystrophy. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21:902–909, 1998.

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Section: Discussionmentioning

confidence: 96%

“…For immunofluorescence labeling, cells were plated at 7 × 10 3 per 35-mm culture dish. Human normal (RCMH) and Duchenne muscular dystrophy (RCDMD) immortal cell lines were cultured as described, 6,7 and the normal murine cell line (129CB3) 30 cells were used as a control.…”

Section: Methodsmentioning

confidence: 99%

Differences in both inositol 1,4,5-trisphosphate mass and inositol 1,4,5-trisphosphate receptors between normal and dystrophic skeletal muscle cell lines

Liberona¹,

Powell²,

Shenoi³

et al. 1998

Muscle Nerve

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“…DMD is an inherited X-linked muscular dystrophy generated due to the mutations in DMD gene, encoding Dystrophin protein. As discussed above, not only one specific mutation but rather combinatorial effects [25,[48][49][50][51][52]. But none of these approaches alone can cure the disease completely, majorly because in Dp malfunctioning or deficiency, not only DAPC mediated mechanical support gets destabilized but also a drastic change occurs in the flux of ion current through the ion channels in cell membrane [44].…”

Section: Biological Implementationsmentioning

confidence: 99%

Domain Wise Distribution of Mutations in Dystrophin Protein and Duchenne Muscular Dystrophy

Bagchi¹

2015

Gene Technol

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Duchenne muscular dystrophy, the most common inherited X-linked recessive muscular dystrophy, affects 20000 new borns per year globally. Since its discovery in 1860, extensive research works have been carried out to understand the complex architecture of the disease formation. Reason behind the onset of the disease has been mapped back to the set of mutations of different types in dystrophin gene (DMD, 2.4 million bp), the largest gene in the body. Dystrophin (Dp), the cytosolic protein acts as the root of the complex which was primarily thought to link extracellular matrix with cellular actin cytoskeleton but later on has been associated with the stability of the cells, signal transduction as well as in proper development. In this review, we gathered the details of all the mutations occurring in DMD gene and observed that majority of the mutations are present in the N terminal Actin Binding Domain. Some of the mutations were found to be present in the Cysteine Rich Domain of the protein, reflecting the point that these two domains are the most mutation prone regions contributing to Duchenne Muscular Dystrophy (DMD) onset. This review therefore gives an integrative view describing the involvements of Dp in regulating the complexity of DMD disease with a future aspect to study the structural details of DMD genes along with its genetic variations.

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“…Both cell lines have been well characterized previously [30,33,34]. In addition, immunofluorescence studies to detect dystrophin expression in RCMH and RCDMD cells have been performed to test possible reversions in either the phenotype or the genotype that could have occurred during several culture passages.…”

Section: Methodsmentioning

confidence: 99%

“…Our results suggest that IP 3 -dependent slow Ca 2+ signals evoked by electrical stimulation stimulate NRG-1β transcription in dystrophic RCDMD cells. Cell culture Dystrophic human myoblasts of the RCDMD cell line [33] and normal human myoblasts RCMH [34] were cultured in DMEM-F12 (1:1) supplemented with 10% fetal bovine serum and 10% horse serum at 37°C and 5% CO 2 . Myogenic differentiation was accomplished by mere horse serum reduction to 1%.…”

Section: Introductionmentioning

confidence: 99%

Electrical Stimulation Induces Calcium-dependent Up-regulation of Neuregulin-1� in Dystrophic Skeletal Muscle Cell Lines

Juretić

Jorquera

Caviedes

et al. 2012

Cell Physiol Biochem

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Duchenne muscular dystrophy (DMD) is a neuromuscular disease originated by reduced or no expression of dystrophin, a cytoskeletal protein that provides structural integrity to muscle fibres. A promising pharmacological treatment for DMD aims to increase the level of a structural dystrophin homolog called utrophin. Neuregulin-1 (NRG-1), a growth factor that potentiates myogenesis, induces utrophin expression in skeletal muscle cells. Microarray analysis of total gene expression allowed us to determine that neuregulin-1β (NRG-1β) is one of 150 differentially expressed genes in electrically stimulated (400 pulses, 1 ms, 45 Hz) dystrophic human skeletal muscle cells (RCDMD). We investigated the effect of depolarization, and the involvement of intracellular Ca²⁺ and PKC isoforms on NRG-1β expression in dystrophic myotubes. Electrical stimulation of RCDMD increased NRG-1β mRNA and protein levels, and mRNA enhancement was abolished by actinomycin D. NRG-1β transcription was inhibited by BAPTA-AM, an intracellular Ca²⁺ chelator, and by inhibitors of IP₃-dependent slow Ca²⁺ transients, like 2-APB, Ly 294002 and Xestospongin B. Ryanodine, a fast Ca²⁺ signal inhibitor, had no effect on electrical stimulation-induced expression. BIM VI (general inhibitor of PKC isoforms) and Gö 6976 (specific inhibitor of Ca²⁺-dependent PKC isoforms) abolished NRG-1β mRNA induction. Our results suggest that depolarization induced slow Ca²⁺ signals stimulate NRG-1β transcription in RCDMD cells, and that Ca²⁺-dependent PKC isoforms are involved in this process. Based on utrophin´s ability to partially compensate dystrophin disfunction, knowledge on the mechanism involved on NRG-1 up-regulation could be important for new therapeutic strategies design.

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Ion channels in a skeletal muscle cell line from a Duchenne muscular dystrophy patient

Cited by 14 publications

References 27 publications

Differences in both inositol 1,4,5-trisphosphate mass and inositol 1,4,5-trisphosphate receptors between normal and dystrophic skeletal muscle cell lines

Differences in both inositol 1,4,5-trisphosphate mass and inositol 1,4,5-trisphosphate receptors between normal and dystrophic skeletal muscle cell lines

Domain Wise Distribution of Mutations in Dystrophin Protein and Duchenne Muscular Dystrophy

Electrical Stimulation Induces Calcium-dependent Up-regulation of Neuregulin-1� in Dystrophic Skeletal Muscle Cell Lines

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