Utrophin is the autosomal homologue of dystrophin. In normal skeletal muscl
e it is localised only to neuromuscular and myotendinous junctions, nerves
and vascular tissue. In Xp21 muscular dystrophies, utrophin is also detecte
d on the sarcolemma of skeletal and cardiac muscle, while dystrophin is abs
ent or reduced. In normal cardiac muscle, some reports have demonstrated ut
rophin at intercalated discs and T-tubules. We have re-examined the distrib
ution of utrophin in normal human cardiac muscle using a panel of eight mon
oclonal antibodies against different epitopes in N- and C-terminal domains.
In contrast to previous studies, utrophin was not detected at the intercal
ated discs or T-tubules, although labelling of blood vessels was strong. We
conclude that the primary location of utrophin in normal heart is in the v
ascular system. In addition, our results show that the utrophin on cardiac
blood vessels is full length, similar to that of skeletal muscle blood vess
els.