| First Author | Chen MF | Year | 1997 |
| Journal | J Physiol | Volume | 504 ( Pt 1) |
| Pages | 75-81 | PubMed ID | 9350619 |
| Mgi Jnum | J:44990 | Mgi Id | MGI:1101568 |
| Doi | 10.1111/j.1469-7793.1997.075bf.x | Citation | Chen MF, et al. (1997) Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage. J Physiol 504(Pt 1):75-81 |
| abstractText | 1. In mature mammalian muscle, the muscular chloride channel ClC-1 contributes about 75% of the sarcolemmal resting conductance (Gm). In mice carrying two defective alleles of the corresponding Clc1 gene, chloride conductance (GCl) is reduced to less than 10% of that of wild-type, and this causes hyperexcitability, the salient feature of the disease myotonia. Potassium conductance (GK) values in myotonic mouse muscle fibres are lowered by about 60% compared with wild-type. 2. The defective Clcadr allele causes loss of the 4.5 kb ClC-1 mRNA. Mice heterozygous for the defective Clc1adr 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 GCl which is not significantly different from that of homozygous wild-type animals. The GK 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. GK is probably indirectly regulated by muscle activity. |
