Utrophin: a potential replacement for dystrophin?
This paper reviews the evidence that utrophin, the autosomally encoded protein related to dystrophin, may be capable of performing the same cellular functions as dystrophin. If this is the case, it may be possible to modify the regulation of utrophin expression as an alternative route to dystrophin gene therapy for sufferers of DMD and/or BMD.
Tinsley
J M
JM
Molecular Genetics Group, John Radcliffe Hospital, Headington, Oxford, U.K.
Davies
K E
KE
D000328:Adult; D000818:Animals; D003598:Cytoskeletal Proteins / Q000096:biosynthesis* / Q000235:genetics; D016189:Dystrophin / Q000096:biosynthesis* / Q000235:genetics; D005314:Embryonic and Fetal Development; D005786:Gene Expression Regulation; D015316:Genetic Therapy; D005865:Gestational Age; D006801:Humans; D008565:Membrane Proteins; D051379:Mice; D008818:Mice, Neurologic Mutants; D024510:Muscle Development; D009132:Muscles / Q000196:embryology / Q000378:metabolism; D009136:Muscular Dystrophies / Q000235:genetics* / Q000378:metabolism / Q000628:therapy*; D009137:Muscular Dystrophy, Animal / Q000378:metabolism; D049411:Utrophin
D003598:Cytoskeletal Proteins; D016189:Dystrophin; D008565:Membrane Proteins; C483600:Utrn protein, mouse; D049411:Utrophin