Defective association of dystrophin with sarcolemmal glycoproteins in the cardiomyopathic hamster heart.
In ventricular muscle from 30- to 60-day-old Bio 14.6 cardiomyopathic hamsters, dystrophin-associated glycoproteins of 43, 50 and 150 kDa are markedly reduced in abundance. In particular, the 50-kDa glycoprotein is totally deficient in the sarcolemma of myopathic ventricular myocytes as revealed by immunofluorescence microscopy. The dystrophin-glycoprotein complex formation is defective in the cardiomyopathic hamster heart, because dystrophin and the glycoproteins behave independently when digitonin-solubilized ventricular homogenates are fractionated on wheat germ agglutinin beads or anti-dystrophin immunoaffinity beads.
Department of Molecular Physiology, National Cardiovascular Center Research Institute, Osaka, Japan.
Y
Y
Iwata
H
H
Nakamura
Y
Y
Mizuno
M
M
Yoshida
E
E
Ozawa
M
M
Shigekawa
D000818:Animals; D009202:Cardiomyopathies / Q000378:metabolism*; D006224:Cricetinae; D004072:Digitonin; D016189:Dystrophin / Q000302:isolation & purification / Q000378:metabolism*; D005455:Fluorescent Antibody Technique; D006023:Glycoproteins / Q000737:chemistry / Q000378:metabolism*; D006352:Heart Ventricles / Q000737:chemistry; D015151:Immunoblotting; D007163:Immunosorbent Techniques; D008647:Mesocricetus; D008970:Molecular Weight; D012508:Sarcolemma / Q000378:metabolism*; D000254:Sodium-Potassium-Exchanging ATPase / Q000378:metabolism; D012995:Solubility; D014909:Wheat Germ Agglutinins
D016189:Dystrophin; D006023:Glycoproteins; D014909:Wheat Germ Agglutinins; D000254:Sodium-Potassium-Exchanging ATPase; D004072:Digitonin