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Photo of Kevin Campbell

Kevin P. Campbell

Professor,  Physiology & Biophysics

Contact Information

Phone: +1 319 335 7867
Email: kevin-campbell@uiowa.edu
Web: Lab Website

Education


Appointments

Primary: Physiology & Biophysics
Secondary: Neurology
Secondary: Internal Medicine

Centers and Program Affiliations


Research Interests

dysferlin, dystroglycan, membrane repair, muscular dystrophy, skeletal muscle

MeSH Terms from Publications

Dystroglycans, Dystrophin, Muscular Dystrophies, Cytoskeletal Proteins, Animals, Membrane Glycoproteins, Calcium Channels, Sarcoglycans, Muscles, Muscle, Skeletal, Rabbits, Laminin, Mice, Muscle Proteins, Sarcoplasmic Reticulum, Muscular Dystrophy, Animal, Sarcolemma, Humans, Molecular Weight, Membrane Proteins, Utrophin, Ryanodine Receptor Calcium Release Channel, Mice, Inbred mdx, Calcium, Glycosylation

Research Summary

Research in my laboratory is focused on elucidating the molecular basis of muscular dystrophy, and developing therapeutic strategies to treat muscular dystrophy. Muscular dystrophies, a group of genetic diseases that primarily affect skeletal muscle, are characterized by progressive muscle weakness. Duchenne muscular dystrophy is caused by mutations in the dystrophin gene that lead to the complete absence of dystrophin in skeletal muscle. Research in my laboratory on the function of dystrophin led to the discovery of the skeletal muscle dystrophin-glycoprotein complex, which spans the muscle cell membrane and links the sub-sarcolemma actin cytoskeleton to the surrounding basement membrane. Defects in genes that encode either components of the complex itself or mediators of its requisite post-translational modifications lead to distinct forms of muscular dystrophy. My current and future research focuses on four related areas: (1) the molecular pathogenesis of dystrophin-glycoprotein complex disorders, (2) the mechanistic basis of maintaining muscle membrane integrity, (3) the molecular pathogenesis of disorders arising from defects in dystroglycan glycosylation, and (4) the structural basis of dystroglycan function as a basement membrane receptor.



Recent Publications


Show publications
  1. Dystroglycan on radial glia end feet is required for pial basement membrane integrity and columnar organization of the developing cerebral cortex. J Neuropathol Exp Neurol 71(12):1047-63, 2012. [PubMed]
  2. Mouse fukutin deletion impairs dystroglycan processing and recapitulates muscular dystrophy. J Clin Invest 122(9):3330-42, 2012. [PubMed]
  3. Binding of Lassa virus perturbs extracellular matrix-induced signal transduction via dystroglycan. Cell Microbiol 14(7):1122-34, 2012. [PubMed]
  4. ISPD loss-of-function mutations disrupt dystroglycan O-mannosylation and cause Walker-Warburg syndrome. Nat Genet 44(5):575-80, 2012. [PubMed]
  5. Dystroglycan function requires xylosyl- and glucuronyltransferase activities of LARGE. Science 335(6064):93-6, 2012. [PubMed]
  6. Endpoint measures in the mdx mouse relevant for muscular dystrophy pre-clinical studies. Neuromuscul Disord 22(1):34-42, 2012. [PubMed]
  7. Combined deficiency of alpha and epsilon sarcoglycan disrupts the cardiac dystrophin complex. Hum Mol Genet 20(23):4644-54, 2011. [PubMed]
  8. Two separate Ni(2+) -sensitive voltage-gated Ca(2+) channels modulate transretinal signalling in the isolated murine retina. Acta Ophthalmol 89(7):e579-90, 2011. [PubMed]
  9. Like-acetylglucosaminyltransferase (LARGE)-dependent modification of dystroglycan at Thr-317/319 is required for laminin binding and arenavirus infection. Proc Natl Acad Sci U S A 108(42):17426-31, 2011. [PubMed]
  10. Anti-epileptic drugs delay age-related loss of spiral ganglion neurons via T-type calcium channel. Hear Res 278(1-2):106-12, 2011. [PubMed]