Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage

Chen MF, Niggeweg R, Iaizzo PA, LehmannHorn F, Jockusch H (1997)
JOURNAL OF PHYSIOLOGY-LONDON 504(1): 75-81.

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1. In mature mammalian muscle, the muscular chloride channel CIC-1 contributes about 75% of the sarcolemmal resting conductance (G(m)). In mice carrying two defective alleles of the corresponding Clc1 gene, chloride conductance (G(Cl)) is reduced to less than 10% of that of wild-type, and this causes hyperexcitability, the salient feature of the disease myotonia. Potassium conductance (G(K)) values is myotonic mouse muscle fibres are lowered by about 60% compared with wild-type. 2. The defective Clc(adr) allele causes loss of the 4.5 kb ClC-1 mRNA. Mice heterozygous for the defective Clc1(adr) allele contain about 50% functional mRNA in their muscles compared. with homozygous wild-type mice. 3. Despite a halved functional gene dosage, heterozygous muscles display an average G(Cl), which is not significantly different from that of homozygous wild-type animals. The G(K) values in heterozygotes are also indistinguishable from homozygous wild-type animals. 4. These results indicate that a regulatory mechanism acting at the post-transcriptional level limits the density of ClC-1 channels. G(K) is probably indirectly regulated by muscle activity.
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Chen MF, Niggeweg R, Iaizzo PA, LehmannHorn F, Jockusch H. Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage. JOURNAL OF PHYSIOLOGY-LONDON. 1997;504(1):75-81.
Chen, M. F., Niggeweg, R., Iaizzo, P. A., LehmannHorn, F., & Jockusch, H. (1997). Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage. JOURNAL OF PHYSIOLOGY-LONDON, 504(1), 75-81.
Chen, M. F., Niggeweg, R., Iaizzo, P. A., LehmannHorn, F., and Jockusch, H. (1997). Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage. JOURNAL OF PHYSIOLOGY-LONDON 504, 75-81.
Chen, M.F., et al., 1997. Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage. JOURNAL OF PHYSIOLOGY-LONDON, 504(1), p 75-81.
M.F. Chen, et al., “Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage”, JOURNAL OF PHYSIOLOGY-LONDON, vol. 504, 1997, pp. 75-81.
Chen, M.F., Niggeweg, R., Iaizzo, P.A., LehmannHorn, F., Jockusch, H.: Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage. JOURNAL OF PHYSIOLOGY-LONDON. 504, 75-81 (1997).
Chen, MF, Niggeweg, R, Iaizzo, PA, LehmannHorn, F, and Jockusch, Harald. “Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage”. JOURNAL OF PHYSIOLOGY-LONDON 504.1 (1997): 75-81.
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