Distribution of dystrophin, nebulin and Ricinus communis I (RCA-I)-binding glycoprotein in tissues of normal and mdx mice.
Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene locus, appeared as an immunoreactive triplet of polypeptides in striated muscle tissues from normal mice on Western blot analysis. In smooth muscle tissues, an immunoreactive doublet of corresponding molecular weight was detected. No dystrophin was found in normal mouse brain, not even after enrichment for the Triton X-100 insoluble fraction. Dystrophin was absent from all corresponding tissues from the mdx mutant mouse strain which is known to lack dystrophin. The possibility that these immunoreactive bands represent isoforms is discussed. We have also investigated two other high molecular weight proteins which show secondary abnormalities in DMD muscle, namely nebulin and the 370 kDa Ricinus communis I lectin (RCA I)-binding glycoprotein. Nebulin levels were reduced in skeletal muscle from 6-week-old mdx mice but not in oesophagus from the same animals. By contrast, the RCA I-binding 370 kDa glycoprotein which is greatly reduced in DMD skeletal muscle was present in normal amounts in mdx skeletal muscle. These findings show, for the first time, that mdx myopathy differs from DMD myopathy not only morphologically, but also in its secondary biochemical abnormalities.
Voit
T
T
Jerry Lewis Muscle Research Centre, Department of Paediatrics and Neonatal Medicine, Hammersmith Hospital, London, U.K.
Patel
K
K
Dunn
M J
MJ
Dubowitz
V
V
Strong
P N
PN
D000818:Animals; D016189:Dystrophin; D037102:Lectins; D008562:Membrane Glycoproteins / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D009124:Muscle Proteins / Q000378:metabolism*; D009132:Muscles / Q000378:metabolism; D009137:Muscular Dystrophy, Animal / Q000378:metabolism*; D037121:Plant Lectins; D014018:Tissue Distribution
D016189:Dystrophin; D037102:Lectins; D008562:Membrane Glycoproteins; D009124:Muscle Proteins; D037121:Plant Lectins; C017707:Ricinus communis agglutinin-1; C035620:nebulin