PMID: 9447606Feb 3, 1998Paper

Dystrophin is replaced by utrophin in frog heart; implications for muscular dystrophy

Neuromuscular Disorders : NMD
G E Morris

Abstract

The possibility of using utrophin upregulation as a treatment for dystrophin-deficient muscular dystrophies has focused attention on the question of how many of dystrophin's various functions can be performed by the closely-related protein, utrophin. In Xenopus heart, little or no dystrophin was found on Western blots but the dystrophin-related protein, utrophin, was abundant. This utrophin was shown by immunofluorescence microscopy to be associated with cardiac muscle membranes and its distribution was similar to that of dystrophin in rabbit heart. The utrophin distribution pattern in the frog heart was shared by beta-dystroglycan, a transmembrane protein responsible for localizing both dystrophin and utrophin at cell membranes. The results suggest that utrophin in Xenopus heart can perform similar functions to dystrophin in mammalian heart, lending further support to the possibility of utrophin upregulation therapy in muscular dystrophy. In skeletal muscle, however, Xenopus resembles mammals in expressing dystrophin at the sarcolemma and very little utrophin.

References

Dec 10, 1992·Nature·J M TinsleyD R Love
Oct 4, 1995·Biochemical and Biophysical Research Communications·G E MorrisT M Nguyen
Apr 26, 1995·Biochemical and Biophysical Research Communications·S HoriG E Morris
Mar 1, 1994·Neuromuscular Disorders : NMD·T R HelliwellG E Morris
Oct 1, 1993·Journal of the Neurological Sciences·Y K HayashiK Arahata
Mar 1, 1993·Journal of Neuropathology and Experimental Neurology·G KarpatiP Holland
Jun 14, 1996·Biochemical and Biophysical Research Communications·G RosaT C Petrucci
Jan 1, 1996·Cell Motility and the Cytoskeleton·M JamesG E Morris
Feb 1, 1997·Circulation Research·S StevensonN J Severs
Dec 1, 1996·Neuromuscular Disorders : NMD·D J Duggan, E P Hoffman
Feb 24, 1997·The Journal of Cell Biology·R M GradyJ R Sanes
Feb 24, 1997·The Journal of Cell Biology·A E DeconinckK E Davies

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Citations

Nov 10, 2000·Current Opinion in Neurology·S FletcherJ M Howell
Feb 19, 2000·Microscopy Research and Technique·F H AndradeH J Kaminski

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