Key result
Heterozygous mice with a halved functional Clc1 gene dosage display average chloride and potassium conductances that are not significantly different from homozygous wild-type animals.
A post-transcriptional regulatory mechanism compensates for a halved Clc1 gene dosage to maintain normal chloride and potassium conductance in mouse muscle.
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No immediate clinical implications for channelopathies; leaves open whether post-transcriptional compensation extends to human muscle or cardiac disorders.
Chen et al. (1997) studied Myotonia. Heterozygous defective Clc1adr allele vs. Homozygous wild-type was evaluated on Chloride conductance (GCl) and potassium conductance (GK). Heterozygous mice with a halved functional Clc1 gene dosage display average chloride and potassium conductances that are not significantly different from homozygous wild-type animals.