Advertisement
Journal of Biological Chemistry
Open access logo
Advanced searchSave search
Research Article| Volume 269, ISSUE 44, P27635-27639, November 04, 1994

Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle.

Open AccessPublished:November 04, 1994DOI:https://doi.org/10.1016/S0021-9258(18)47032-9
Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle.
Previous ArticleInduction of 15-lipoxygenase by interleukin-13 in human blood monocytes.
Next ArticleIn vitro complex formation between the octamer of mitochondrial creatine kinase and porin.
      This paper is only available as a PDF. To read, Please Download here.
      The muscular chloride channel (ClC-1) is essential for a normal excitability of mature mammalian muscle fibers; inactivation of the corresponding gene by mutations leads to hyperexcitability of muscle, the hallmark of the disease myotonia. In the mouse, there is very little ClC-1 mRNA in myotubes, and its concentration increases steeply during postnatal development, suggesting a role of the motor nerve in ClC-1 expression. We investigated the response of the expression of the corresponding gene Clc-1 to different patterns of muscle activity as controlled by sarcolemmal excitability and by innervation. In rat and mouse, the level of ClC-1 mRNA was higher in fast (extensor digitorum longus) than in slow (soleus) muscle. Myotonia in the ADR mouse is caused by an insertional mutation leading to the adr allele of the Clc-1 gene and to grossly abnormal ClC-1 mRNAs. Nevertheless, in +/adr heterozygous, phenotypically wild type (WT) animals, the expression levels of both alleles correspond to the gene dosage. However, in the myotonic ADR mouse in which both Clc-1 genes are defective, ClC-1 mRNA levels in slow muscle were nearly as high as in WT fast muscle. In WT muscle, denervation within 2 days caused a drastic reduction of the ClC-1 mRNA level and at the same time an increase of myogenin and MyoD mRNAs. Neither effect of denervation was observed in myotonic mice (homozygous for the alleles adr or adrK), suggesting that spontaneous electrical activity of the hyperexcitable sarcolemma may substitute for nerve activity. Furthermore, potential MyoD/myogenin-binding sequence motifs were identified in the 5' regulatory region of the Clc-1 gene. These findings suggest that the activity-dependent regulation of the muscular chloride channel 1 gene is mediated by myogenic factors.

      REFERENCES

        • Barton P.J.R.
        • Cohen A.
        • Robert B.
        • Fiszman M.Y.
        • Bonhomme F.
        • Guénet J.-L.
        • Leader D.P.
        • Buckingham M.E.
        J. Biol. Chem. 1985; 260: 8578-8584
        • Bassel-Duby R.
        • Hernandez M.D.
        • Gonzalez M.A.
        • Krueger J.K.
        • Williams R.S.
        Mol. Cell. Biol. 1992; 12: 5024-5032
        • Blackwell T.K
        • Huang J.
        • Ma A.
        • Kretzner L.
        • Alt F.W.
        • Eisenman R.N.
        • Weintraub H.
        Mol. Cell. Biol. 1993; 13: 5216-5224
        • Bretag A.H.
        Physiol. Rev. 1987; 67: 618-724
        • Chirgwin J.M.
        • Przybyla A.E.
        • MacDonald R.J.
        • Rutter W.J.
        Biochemistry. 1979; 18: 5294-5299
        • Chomczynski P.
        • Sacchi N.
        Anal. Biochem. 1987; 162: 156-159
        • Church G.M.
        • Gilbert W.
        Proc. Natl. Acad. Sci. U. S. A. 1984; 81: 1991-1995
        • Conte Camerino D.
        • De Luca A.
        • Mambrini M.
        • Vrbova G.
        Pfliigers Arch. Eur. J. Physiol. 1989; 413: 568-570
        • Davis R.L.
        • Weintraub H.
        • Lassar A.B.
        Cell. 1987; 51: 987-1000
        • Edmondson D.G.
        • Olson E.N.
        Genes & Dev. 1989; 3: 628-640
        • Eftimie R.
        • Brenner H.R.
        • Buonanno A.
        Proc. Natl. Acad. Sci. U. S. A. 1991; 88: 1349-1353
        • Evans S.
        • Goldman D.
        • Heinemann S.
        • Patrick J.
        J. Biol. Chem. 1987; 262: 4911-4916
        • Fakler B.
        • Ruppersberg J.P.
        • Spittelmeister W.
        • Riidel R.
        Pfliigers Arch. Eur. J. Physiol. 1990; 415: 693-700
        • Feinberg A.P.
        • Vogelstein B.
        Anal. Biochem. 1983; 132: 6-13
        • Füchtbauer E.-M.
        • Rowlerson A.M.
        • Götz K.
        • Friedrich G.
        • Mabuchi K.
        • Gergely J.
        • Jockusch H.
        J. Histochem. Cytochem. 1991; 39: 355-361
        • George Jr., A.L.
        • Crackover M.A.
        • Abdalla J.A.
        • Hudson A.J.
        • Ebers G.C.
        Nature Genet. 1993; 3: 305-310
        • Goblet C.
        • Whalen R.G.
        J. Cell. Biochem. 1994; 18: 528
        • Goldman D.
        • Brenner H.R.
        • Heinemann S.
        Neuron. 1988; 1: 329-333
        • Gronemeier M.
        • Condie A.
        • Prosser J.
        • Steinmeyer K.
        • Jentsch T.J.
        • Jockusch H.
        J. Biol. Chem. 1994; 269: 5963-5967
        • Gunning P.
        • Ponte P.
        • Okayama H.
        • Engel J.
        • Blau H.
        • Kedes L.
        Mol. Cell. Biol. 1983; 3: 787-795
        • Horlick R.A.
        • Benfield P.A.
        Mol. Cell. Biol. 1989; 9: 2396-2413
        • Hughes S.M.
        • Taylor J.M.
        • Tapscott S.J.
        • Gurley C.M
        • Carter W.J.
        • Peterson C.A.
        Development (Camb.). 1993; 118: 1137-1147
        • Isenberg K.E.
        • Mudd J.
        • Shah V.
        • Merlie J.P.
        Nucleic Acids Res. 1986; 14: 5111
        • Jockusch H.
        Pette D. in The Dynamic State of Muscle Fibers. Walter de Gruyter and Co., Berlin1990: 429-443
        • Jockusch H.
        • Reininghaus J.
        • Stuhlfauth I.
        • Zippel M.
        Eur. J. Biochem. 1988; 171: 101-105
        • Jockusch H.
        • Kaupmann K.
        • Gronemeier M.
        • Schleef M.
        • Klocke R.
        Prog. Neurobiol. 1994; 42: 313-317
        • Jockusch H.
        • Gronemeier M.
        • Condie A.
        • Prosser J.
        • Jentsch T.J.
        • Steinmeyer K.
        • Klocke R.
        J. Cell. Biochem. 1994; 18: 528
        • Klocke R.
        Expression and Chromosomale Lokalisation von Ionenkanal-genen der Maus. Ph.D. thesis. University of Bielefeld, Germany1994
        • Kluxen F.-W.
        • Schöffl F.
        • Berchtold M.W.
        • Jockusch H.
        Eur. J. Biochem. 1988; 176: 153-158
        • Koch M.C.
        • Steinmeyer K.
        • Lorenz C.
        • Ricker K.
        • Wolf F.
        • Otto M.
        • Zoll B.
        • Lehmann-Horn F.
        • Grzeschik K.-H.
        • Jentsch T.J.
        Science. 1992; 257: 797-800
        • Kroczek R.A.
        • Siebert E.
        Anal. Biochem. 1990; 184: 90-95
        • Laskey R.A.
        • Mills A.D.
        FEBS Lett. 1977; 82: 314-316
        • Lassar A.B.
        • Buskin J.N.
        • Lockshon D.
        • Davis R.L.
        • Apone S.
        • Hauschka S.D.
        • Weintraub H.
        Cell. 1989; 58: 823-831
        • Lorenz C.
        • Meyer-Kleine C.
        • Steinmeyer K.
        • Koch M.C.
        • Jentsch T.J.
        Hum. Mol. Genet. 1994; 3: 941-946
        • Lorkovic H.
        • Tomanek R.J.
        Am. J. Physiol. 1977; 232: C109-C114
        • Lyons G.E.
        • Mühlebach S.
        • Moser A.
        • Masood R.
        • Paterson B.M.
        • Buckingham M.E.
        • Perriard J.-C.
        Development (Camb.). 1991; 113: 1017-1029
        • Mehrke G.
        • Brinkmeier H.
        • Jockusch H.
        Muscle & Nerve. 1988; 11: 440-446
        • Pette D.
        • Vrbová G.
        Muscle & Nerve. 1985; 8: 676-689
        • Piette J.
        • Bessereau J.L.
        • Huchet M.
        • Changeux J.P.
        Nature. 1990; 345: 353-355
        • Sanes J.R.
        • Johnson Y.R.
        • Kotzbauer P.T.
        • Mudd J.
        • Hanley T.
        • Martinou J.-C.
        • Merlie J.P.
        Development. 1991; 113: 1181-1191
        • Schleef M.
        Erbliche Myotonie der Maus: Molekulargenetische Analysen Deregulierter Gene undlhrer Transkripte. Ph.D. thesis. University of Bielefeld, Germany1992
        • Schleef M.
        • Zühlke C.
        • Schöffl F.
        • Jockusch H.
        Neuromuscular Disorders. 1994; 4: 205-217
        • Steinmeyer K.
        • Klocke R.
        • Ortland C.
        • Gronemeier M.
        • Jockusch H.
        • Gründer S.
        • Jentsch T.J.
        Nature. 1991; 354: 304-308
        • Steinmeyer K.
        • Ortland C.
        • Jentsch T.J.
        Nature. 1991; 354: 301-304
        • Steinmeyer K.
        • Lorenz C.
        • Pusch M.
        • Koch M.C.
        • Jentsch T.J.
        EMBO J. 1994; 13: 737-743
        • Stuhlfauth I.
        • Reininghaus J.
        • Jockusch H.
        • Heizmann C.W.
        Proc. Natl. Acad. Sci. U. S. A. 1984; 81: 4814-4818
        • Wang X.-M.
        • Tsay H.-J.
        • Schmidt J.
        EMBO J. 1990; 9: 783-790
        • Weller P.A.
        • Price M.
        • Isenberg H.
        • Edwards Y.H.
        • Jeffreys A.J.
        Mol. Cell. Biol. 1986; 6: 4539-4547
        • Wischmeyer E.
        • Nolte E.
        • Klocke R.
        • Jockusch H.
        • Brinkmeier H.
        Neuromuscular Disorders. 1993; 3: 267-274
        • Witzemann V.
        • Barg B.
        • Criado M.
        • Stein E.
        • Sakmann B.
        FEBS Lett. 1989; 242: 419-424
        • Witzemann V.
        • Stein E.
        • Barg B.
        • Konno T.
        • Koenen M.
        • Kues W.
        • Criado M.
        • Hofmann M.
        • Sakmann B.
        Eur. J. Biochem. 1990; 194: 437-448
        • Witzemann V.
        • Brenner H.-R.
        • Sakmann B.
        J. Cell Biol. 1991; 114: 125-140
        • Zühlke C.
        • Schöffl F.
        • Jockusch H.
        • Simon D.
        • Guénet J.-L.
        Genet. Res. 1989; 54: 37-43

      Article info

      Publication history

      Published online: November 04, 1994

      Identification

      DOI: https://doi.org/10.1016/S0021-9258(18)47032-9

      Copyright

      © 1994 ASBMB. Currently published by Elsevier Inc; originally published by American Society for Biochemistry and Molecular Biology.

      User license

      Creative Commons Attribution (CC BY 4.0) |
      How you can reuse Information Icon
      Creative Commons Attribution (CC BY 4.0)

      Permitted

      • Read, print & download
      • Redistribute or republish the final article
      • Text & data mine
      • Translate the article
      • Reuse portions or extracts from the article in other works
      • Sell or re-use for commercial purposes

      Elsevier's open access license policy

      ScienceDirect

      Access this article on ScienceDirect

      ASBMB  ASBMB  ASBMB  ASBMB

      ISSN 0021-9258
      We use cookies to help provide and enhance our service and tailor content. To update your cookie settings, please visit the for this site.
      Copyright © 2022 Elsevier Inc. except certain content provided by third parties. The content on this site is intended for healthcare professionals.


      RELX