Penn Medicine

Simona Boncompagni, Ph.D.

Cell and Developmental Biology

Simona Boncompagni

Contact Information

University of Pennsylvania School of Medicine
Philadelphia, PA 19104-6058
Phone: (215) 898-3345
Fax: (215) 573-2170


Research Summary

a - CRUs-mitochondria interaction  in developing and mature skeletal muscle fibers.
b - EC-coupling and skeletal muscle disease.
c – Role of the EC coupling apparatus in age-related decline of skeletal muscle performance.
Figure Tethers.jpg

Selected Publications

  1. Boncompagni, S., L. d’Amelio, S. Fulle, G. Fanò, and F. Protasi. 2006. Progressive disorganization of the excitation-contraction coupling apparatus in ageing human skeletal muscle as revealed by electron microscopy: a possible role in the decline of muscle performance.  J. Gerontol. Biol. Sci. 61:995-1008.
  2. Boncompagni, S., H. Kern, K. Rossini, W. Mayr, U. Carraro, and F. Protasi. 2007. Structural differentiation of skeletal muscle fibers in absence of innervation in humans. Proc. Natl. Acad. Sci. USA. 104:19339-19344.
  3. Durham, W. J., P. Aracena-Parks, C. Long, A. E. Rossi, S. A. Goonasekera, S. Boncompagni, D. L. Galvan, C. P. Gilman,  N. Shirokova,  F. Protasi,  R. T. Dirksen, and S. L. Hamilton. 2008. RYR1 S- Nitrosilation underlies environmental heat stroke and sudden death in Y522S RyR1 knockin mice. Cell. 133:53-65.
  4. Boncompagni, S., A. E. Rossi, M. Micaroni, G. V. Beznoussenko, R. S. Polishchuk, R. T. Dirksen, and F. Protasi. 2009. Mitochondria are linked to calcium stores in striated muscle by developmentally regulated tethering structures.  Mol. Biol. Cell. 20:1058-1067.
  5. Boncompagni, S., A. E. Rossi, M. Micaroni, S. L. Hamilton, R. T. Dirksen, C. Franzini-Armstrong, and F. Protasi. 2009b. Characterization and temporal development of cores in a mouse model of malignant hyperthermia. Proc. Natl. Acad. Sci. USA. 106:21996-22001.
  6. Boncompagni, S., E. L. Ryan, R. T. Dirksen and C. Franzini-Armstrong. 2010. The I4895T mutation in the type 1 ryanodine receptor induces fiber-type specific alterations in skeletal muscle that mimic premature aging. Aging Cell. 9:958:970.
  7. Boncompagni, S., F. Protasi, and C. Franzini-Armstrong. Sequential stages in the aged-dependent gradual formation and accumulation of tubular aggregates in aging fast twitch muscle: SERCA and Calsequestrin Involvement. Age. (Dordr). 2011 Feb 12.


Pennsylvania Muscle Institute
Perelman School of Medicine University of Pennsylvania
Director: E. Michael Ostap, Ph.D.

700A Clinical Research Building Philadelphia, PA 19104-6085 Phone: (215) 573-9758 Fax: (215) 898-2653