The dystrophin-related protein, utrophin, is expressed on the sarcolemma of regenerating human skeletal muscle fibres in dystrophies and inflammatory myopathies.
Utrophin is the 400 kDa protein product of an autosomal homologue (DMDL) of the dystrophin gene. In normal skeletal muscle, utrophin is expressed in vascular smooth muscle, endothelium and nerves but not in mature muscle fibres except at the neuromuscular junction. We have examined the expression of utrophin in a wide range of human skeletal muscle diseases using monoclonal antibodies against three C-terminal epitopes. Utrophin is consistently expressed in all basophilic, regenerating fibres irrespective of the underlying disease or expression of dystrophin. It is also found in regenerating fibres from a normal volunteer. In Duchenne and Becker dystrophies, as well as in dermatomyositis, sarcolemmal staining for utrophin is also seen in larger fibres which are not obviously regenerating. These studies do not support the idea that utrophin occupies membrane attachment sites only when dystrophin is absent or reduced, but would be consistent with utrophin expression as part of an activated foetal programme during regeneration.
Helliwell
T R
TR
Department of Pathology, University of Liverpool, U.K.
Man
N T
NT
Morris
G E
GE
Davies
K E
KE
D000293:Adolescent; D000328:Adult; D000911:Antibodies, Monoclonal; D002648:Child; D002675:Child, Preschool; D003598:Cytoskeletal Proteins / Q000096:biosynthesis*; D016189:Dystrophin / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D066298:In Vitro Techniques; D007223:Infant; D007249:Inflammation / Q000378:metabolism; D008565:Membrane Proteins; D008875:Middle Aged; D009132:Muscles / Q000294:innervation / Q000378:metabolism* / Q000473:pathology; D009135:Muscular Diseases / Q000378:metabolism* / Q000473:pathology; D009136:Muscular Dystrophies / Q000378:metabolism* / Q000473:pathology; D009416:Nerve Regeneration / Q000502:physiology*; D012508:Sarcolemma / Q000378:metabolism*; D049411:Utrophin
D000911:Antibodies, Monoclonal; D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; D049411:Utrophin