Opposite regulation of the mRNAs for parvalbumin and p19/6.8 in myotonic mouse muscle.
The gene mutation in the mouse, 'arrested development of righting response', adr, causes a defect of chloride conductance of the muscle fibre membrane leading to the symptoms of myotonia [Mehrke, G., Brinkmeier, H. and Jockusch, H. (1988) Muscle & Nerve 11, 440-446]. In fast muscle, the myotonic phenotype is accompanied by a drastic reduction of the Ca2+-binding protein, parvalbumin. Messenger RNA levels in organs of myotonic (ADR) mice were analysed. In fast muscles of the mutant, in-vitro-translatable parvalbumin mRNA was strongly reduced, whereas the mRNA for the slow-muscle-specific protein, p19/6.8, was increased. In contrast, the parvalbumin mRNA in the cerebellum was not affected by the adr mutation. A reduction of the two parvalbumin mRNA species (700 and 1100 nucleotides) in ADR fast muscle and unaltered parvalbumin mRNA levels in mutant cerebella were demonstrated by cDNA/mRNA hybridisation, using a rat parvalbumin cDNA as a probe. The mRNA level for another Ca2+-binding protein, calmodulin, was low in muscle and high in the central nervous system but was unaffected by the mutation. When adr/adr mice were fed a diet containing the membrane-stabilising drug, tocainide, the levels in muscle of the mRNAs for parvalbumin and p19/6.8 were partially normalised.
D000818:Animals; D004591:Electrophoresis, Polyacrylamide Gel; D005786:Gene Expression Regulation; D007120:Immunochemistry; D008012:Lidocaine / Q000031:analogs & derivatives / Q000494:pharmacology; D051379:Mice; D008817:Mice, Mutant Strains; D009124:Muscle Proteins / Q000235:genetics*; D009132:Muscles / Q000378:metabolism*; D009222:Myotonia / Q000235:genetics / Q000378:metabolism*; D009693:Nucleic Acid Hybridization; D010320:Parvalbumins / Q000096:biosynthesis / Q000235:genetics*; D014176:Protein Biosynthesis; D012333:RNA, Messenger / Q000378:metabolism*; D016677:Tocainide
D009124:Muscle Proteins; D010320:Parvalbumins; D012333:RNA, Messenger; C057153:muscle protein p19-6.8; D016677:Tocainide; D008012:Lidocaine