Human Molecular Genetics, Vol 8, 593-599, Copyright © 1999 by Oxford University Press
CN Lumeng, SF Phelps, JA Rafael, GA Cox, TL Hutchinson, CR Begy, E Adkins, R Wiltshire and JS Chamberlain
Utrophin is a 400 kDa autosomal homolog of dystrophin and a component of
the submembranous cytoskeleton. While multiple dystrophin isoforms have
been identified along with alternatively spliced products, to date only two
different mRNA species of utrophin have been identified. To determine the
degree of evolutionary conservation between dystrophin and utrophin
isoforms, we have compared their expression patterns in adult mice.
Northern blot analysis of multiple adult tissues confirmed that only two
major sizes of transcripts are produced from each gene: 13 and 5.5 kb from
utrophin and 14 and 4.8 kb from dystrophin. However, western blot analysis
detected several putative short utrophin isoforms that may be homologs of
the dystrophin isoforms Dp140, Dp116 and Dp71. We also identified an
alternatively spliced utrophin transcript that lacks the equivalent of the
alternatively spliced dystrophin exon 71. Finally, we demonstrated that the
C-terminal domain of utrophin targeted to neuromuscular junctions in normal
mice, but localized to the sarcolemma efficiently only in the absence of
dystrophin. Our results provide further evidence for a common evolutionary
origin of the utrophin and dystrophin genes.
ARTICLES
Characterization of dystrophin and utrophin diversity in the mouse
Department of Human Genetics, University of Michigan Medical School, 1150 West Medical Center Drive, Ann Arbor, MI 48109-0618, USA.
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