Steven A. Moore M.D., Ph.D.
steven-mooreatuiowadotedu
Professor of Pathology

Basic science collaboration with Dr. Kevin Campbell, Department of Physiology and Biophysics involves the pathologic characterization of genetic mouse models of muscular dystrophy. Our most recent basic science collaborations use Cre-lox methodology to selectively knock out brain or peripheral nerve dystroglycan. These mice model congenital muscular dystrophy. Clinical diagnostic work in the general area of muscular dystrophies has expanded into basic and clinical research projects in collaboration with Drs. Jerry Mendell (Department of Neurology, Ohio State University), Kevin Campbell, and Kathy Mathews (Department of Pediatrics, The University of Iowa). Current clinical studies involve: (1) a multicenter study of limb-girdle muscular dystrophy (LGMD) aimed at characterizing genotype/phenotype relationships in this diverse array of muscular dystrophies, (2) a gentamicin treatment trial in Duchenne and sarcoglycan-deficient muscular dystrophy patients with mutations that lead to stop codons, and (3) a safety trial of AAV-minidystrophin in Duchenne muscular dystrophy patients.

http://www.medicine.uiowa.edu/pathology/path_folder/
research/moore/index.html

Selected Publications

Cohn RC, Durbeej M, Moore SA, Coral-Vazquez R, Prouty S and Campbell KP. Prevention of cardiomyopathy in mouse models lacking the smooth muscle sarcoglycan-sarcospan complex. J Clin Invest 107:R1-R7, 2001.

Michele DE, Barresi R, Kanagawa M, Saito F, Cohn RD, Satz JS, Dollar J, Nishino I, Kelley RI, Somer H, Straub V, Mathews KD, Moore SA, and Campbell KP. Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies. Nature 418:417-422, 2002.

Moore SA, Saito F, Chen J, Michele DE, Henry MD, Messing A, Cohn RD, Ross-Barta SE, Westra S, Williamson RA, Hoshi T, and Campbell KP. Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy. Nature 418:422-425, 2002.

Cohn RD, Henry MD, Barresi R, Michele DE, Barresi R, Saito F, Moore SA, Flanagan JD, Skwarchuk MW, Robbins ME, Williamson R, Campbell KP. Disruption of Dag1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration. Cell 110:639-648, 2002.

Saito F, Moore SA, Barresi R, Henry MD, Messing A, Ross-Barta SE, Cohn RD, Williamson RA, Sluka KA, Sherman DL, Brophy PJ, Schmelzer JD, Low PA, Wrabetz L, Feltri ML, Campbell KP. Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel clustering. Neuron 38:747-758, 2003.