Expression of chloride channel 1 mRNA in cultured myogenic cells: a marker of myotube maturation

Bardouille, Claudia; Vullhorst, Detlef; Jockusch, Harald

doi:10.1016/0014-5793(96)01098-8

Cited by 21 publications

(18 citation statements)

References 20 publications

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“…However, these studies were performed in cultured myotubes, in which expression of CLCN1 is 100-fold lower than in mature muscle fibers (2). To address this question in mature muscle fibers, we used quantitative RT-PCR to examine levels of mRNA and pre-mRNA for CLCN1 in HSA LR transgenic mice.…”

Section: Resultsmentioning

confidence: 99%

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Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1

Lueck¹,

Lungu²,

Mankodi³

et al. 2007

American Journal of Physiology-Cell Physiology

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Lueck JD, Lungu C, Mankodi A, Osborne RJ, Welle SL, Dirksen RT, Thornton CA. Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1. Am J Physiol Cell Physiol 292: C1291-C1297, 2007. First published November 29, 2006; doi:10.1152/ajpcell.00336.2006.-Transmembrane chloride ion conductance in skeletal muscle increases during early postnatal development. A transgenic mouse model of myotonic dystrophy type 1 (DM1) displays decreased sarcolemmal chloride conductance. Both effects result from modulation of chloride channel 1 (CLCN1) expression, but the respective contributions of transcriptional vs. posttranscriptional regulation are unknown. Here we show that alternative splicing of CLCN1 undergoes a physiological splicing transition during the first 3 wk of postnatal life in mice. During this interval, there is a switch to production of CLCN1 splice products having an intact reading frame, an upregulation of CLCN1 mRNA encoding full-length channel protein, and an increase of CLCN1 function, as determined by patch-clamp analysis of single muscle fibers. In a transgenic mouse model of DM1, however, the splicing transition does not occur, CLCN1 channel function remains low throughout the postnatal interval, and muscle fibers display myotonic discharges. Thus alternative splicing is a posttranscriptional mechanism regulating chloride conductance during muscle development, and the chloride channelopathy in a transgenic mouse model of DM1 results from a failure to execute a splicing transition for CLCN1.chloride channel 1; nonsense-mediated decay; alternative splicing; CUG repeats; developmental regulation; muscular dystrophy; ion channel SKELETAL MUSCLE UNDERGOES extensive remodeling during early postnatal development. For example, the transverse tubule system (TTS) is a rudimentary structure in neonatal rodents. The TTS develops rapidly after birth and establishes normal spatial relationships with the sarcomere and sarcoplasmic reticulum by the age of 3 wk (14,26).Development of the TTS is temporally coupled with upregulation of sarcolemmal chloride (Cl) conductance (11,40). If this upregulation does not occur, as in individuals with hereditary myotonia due to null mutations in chloride channel 1 (CLCN1), then potassium accumulation in the TTS during muscle activation leads to membrane depolarization, triggering repetitive action potentials (reviewed by Cannon, Ref. 8). CLCN1 is the main carrier of Cl current in skeletal muscle (10). Postnatal upregulation of Cl conductance is associated with an increase of CLCN1 mRNA (37, 40), but it is not known whether this increase is regulated at a transcriptional or posttranscriptional level.Myotonic dystrophy type 1 (DM1) is the most prevalent degenerative disease of skeletal muscle. DM1 is characterized by hereditary myotonia and reduced expression of CLCN1 (9, 28). However, the mutation is in a gene, DMPK, that encodes a serine/threonine protein kinase, and the effect on CLCN1 is indirect. The genetic lesion in DM1 is an exp...

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Section: Resultsmentioning

confidence: 99%

“…Levels of CLCN1 mRNA increase ϳ10 4 -fold in the transition from myoblasts to mature muscle (2). However, CLCN1 transcripts in late fetal and neonatal muscle are mainly relegated by alternative splicing to a "discard" pathway.…”

Section: Discussionmentioning

confidence: 99%

Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1

Lueck¹,

Lungu²,

Mankodi³

et al. 2007

American Journal of Physiology-Cell Physiology

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“…The muscle-specific chloride channel (ClC-1), encoded by CLCN-1 gene, is the predominant chloride channel in adult skeletal muscle (Bardouille et al 1996;Pusch 2002), and loss of function mutations in this gene results in inherited myotonias in humans and other mammals (Beck et al 1996;Koch et al 1992;Rhodes et al 1999;Zhang et al 2000). Aberrant splicing of the CLCN-1 pre-mRNA results in the loss of CIC-1 protein in skeletal muscle of individuals with DM 1 or DM 2 due to introduction of premature termination codons, which is thought to trigger nonsense mediated decay, resulting in degradation of CLCN-1 mRNA (Charlet-B.…”

Section: Clc-1mentioning

confidence: 99%

Misregulation of Alternative Splicing Causes Pathogenesis in Myotonic Dystrophy

Kuyumcu-Martinez

Cooper

2006

Alternative Splicing and Disease

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Myotonic dystrophy (DM), the most common form of adult onset muscular dystrophy, affects skeletal muscle, heart, and the central nervous system (CNS). Mortality results primarily from muscle wasting and cardiac arrhythmias. There are two forms of the disease: DM 1 and DM 2. DM 1, which constitutes 98% of cases, is caused by a CTG expansion in the 3′ untranslated region (UTR) of the DMPK gene. DM 2 is caused by a CCTG expansion in the first intron of the ZNF9 gene. RNA containing CUG-or CCUG-expanded repeats are transcribed but are retained in the nucleus in foci. Disease pathogenesis results primarily from a gain of function of the expanded RNAs, which alter developmentally regulated alternative splicing as well as pathways of muscle differentiation. The toxic RNA has been implicated in sequestration of splicing regulators and transcription factors thereby causing specific symptoms of the disease. Here we review the proposed mechanisms for the toxic effects of the expanded repeats and discuss the molecular mechanisms of splicing misregulation and disease pathogenesis.

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“…On the other hand, mechanical stretch may also cause conspicuous injury or even apoptosis to skeletal muscle [Tan et al, 2009;Sun et al, 2016]. Under specific circumstances, inhibition of apoptosis, like induction of proliferation, of myoblasts is an important mechanism for regeneration of skeletal muscle [Bardouille et al, 1996].…”

Section: Introductionmentioning

confidence: 99%

“…The various effects of mechanical stretch on skeletal muscle are considered to result from disparities in the duration, magnitude, and frequency of the stretch [Bardouille et al, 1996;Song et al, 2012;Chen et al, 2016]. Generally, a skeletal muscle cell is activated to proliferate if the mechanical load is appropriate.…”

Section: Introductionmentioning

confidence: 99%

Simultaneous Study of Mechanical Stretch-Induced Cell Proliferation and Apoptosis on C2C12 Myoblasts

Feng

Tian

Sun

et al. 2018

Cells Tissues Organs

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Mechanical stretch may cause myoblasts to either proliferate or undergo apoptosis. Identifying the molecular events that switch the fate of a stretched cell from proliferation to apoptosis is practically important in the field of regenerative medicine. A recent study on vascular smooth muscle cells illustrated that identification of these events may be achieved by addressing the stretch-induced opposite cellular outcomes simultaneously within a single investigation. To define conditions or a model in which both proliferation and apoptosis can be studied at the same time, we exposed in vitro cultured C2C12 myoblasts to a cyclic mechanical stretch regimen of 15% elongation at a stretching frequency of 1 Hz for 0, 2, 4, 6, or 8 h every day, consecutively, for 3 days. Both proliferation and apoptosis were observed. Moreover, as the duration of the stretch was prolonged, cell proliferation increased until it peaked at the optimal stretching duration. Afterwards, apoptosis gradually prevailed. Therefore, we established a model in which stretch-induced cell proliferation and apoptosis can be studied simultaneously.

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Expression of chloride channel 1 mRNA in cultured myogenic cells: a marker of myotube maturation

Cited by 21 publications

References 20 publications

Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1

Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1

Misregulation of Alternative Splicing Causes Pathogenesis in Myotonic Dystrophy

Simultaneous Study of Mechanical Stretch-Induced Cell Proliferation and Apoptosis on C2C12 Myoblasts

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