Federica Montanaro, Ph.D. :: Nationwide Children's Hospital, Columbus, Ohio

Federica Montanaro, Ph.D.

Federica  Montanaro, Ph.D.

Center for Gene Therapy
Principal Investigator

Principal Investigator

Neurology Residency

Neuromuscular Disorders
Principal Investigator

Principal Investigator

Contact Information

The Research Institute at Nationwide Children's Hospital
700 Childrens Drive
Columbus, OH 43205 [ map ]
PH: (614) 355-3571
FX: (614) 355-5247
Email Me


Federica Montanaro, Ph.D., is a principal investigator in the Center for Gene Therapy, an Assistant Professor of Pediatrics at The Ohio State University College of Medicine, and an adjunct Assistant Professor in the Department of Physiology and Cell Biology at The Ohio State University. Dr. Montanaro’s research focuses on the pathological mechanisms leading to cardiac failure and muscle fibrosis in Duchenne muscular dystrophy.

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  • Female

Languages Spoken:

  • English

Research Interests

Research Center:

Areas of Interest:

  • My laboratory is interested in understanding the pathological mechanisms underlying Duchenne Muscular Dystrophy (DMD). DMD is caused by mutations in the gene coding for dystrophin. Dystrophin is expressed at the membrane of muscle fibers and cardiac cells. Loss of dystrophin leads to chronic muscle damage and eventually muscle is replaced by fibrotic tissue. Patients succumb to heart or respiratory failure before their third decade of life.

    Our laboratory is pursuing two research project. In project 1, we want to understand how loss of dystrophin leads to muscle degeneration and why some muscles are more affected than others. We have developed a proteomics-based protocol to isolate dystrophin from small amounts of muscle and then identify its interacting proteins. We have found new proteins that interact with dystrophin and provide new leads on its muscle-type specific functions. In Project 2, we want to understand the role of interstitial stem cells in the progression of muscle loss in DMD. We have found that stem cells from wild type muscle spontaneously differentiate into muscle. However, when we isolate these same stem cells from dystrophic muscle, only fibrotic cells are formed. We are currently studying pathways that regulate cell-fate choices in these stem cells with the goal of identifying compounds that can re-direct them to form muscle in dystrophic muscle.

Education and Training

Undergraduate School

  • McGillUniveristy
    Date Completed: 06/30/1991

Post Doctoral

  • McGill University
    Date Completed: 06/30/1999

Professional Experience


  • Assistant Professor, Department of Pediatrics, The Research Institute at Nationwide Children's Hospital and Ohio State University


  • HHMI Post-doctoral fellow, Harvard Medical School and Children's Hospital


  • Vulin,Adeline; Wein,Nicolas; Strandjord,Dana,M; Johnson,Eric,K; Findlay,Andrew,R; Maiti,Baijayanta; Howard,Michael,T; Kaminoh,Yuuki,J; Taylor,Laura,E; Simmons,Tabatha,R; Ray,Will,C; Montanaro,Federica; Ervasti,Jim,M; Flanigan,Kevin,M. 2014. The ZZ Domain of Dystrophin in DMD: Making Sense of Missense Mutations.  HUMAN MUTATION. Vol. 35, no. 2. (February): 257-264.
  • He,Wei,A; Berardi,Emanuele; Cardillo,Veronica,M; Acharyya,Swarnali; Aulino,Paola; Thomas-Ahner,Jennifer; Wang,Jingxin; Bloomston,Mark; Muscarella,Peter; Nau,Peter; Shah,Nilay; Butchbach,Matthew,ER; Ladner,Katherine; Adamo,Sergio; Rudnicki,Michael,A; Keller,Charles; Coletti,Dario; Montanaro,Federica; Guttridge,Denis,C. 2013. NF-kappa B-mediated Pax7 dysregulation in the muscle microenvironment promotes cancer cachexia.  JOURNAL OF CLINICAL INVESTIGATION. Vol. 123, no. 11. (November): 4821-4835.
  • Johnson,Eric,K; Li,Bin; Yoon,Jung,Hae; Flanigan,Kevin,M; Martin,Paul,T; Ervasti,James; Montanaro,Federica. 2013. Identification of New Dystroglycan Complexes in Skeletal Muscle.  PLOS ONE. Vol. 8, no. 8. (August): ee73224.
  • Penton,Christopher,M; Thomas-Ahner,Jennifer,M; Johnson,Eric,K; McAllister,Cynthia; Montanaro,Federica. 2013. Muscle Side Population Cells from Dystrophic or Injured Muscle Adopt a Fibro-Adipogenic Fate.  PLOS ONE. Vol. 8, no. 1. (January): ee54553.
  • Yoon,Jung,Hae; Johnson,Eric; Xu,Rui; Martin,Laura,T; Martin,Paul,T; Montanaro,Federica. 2012. Comparative Proteomic Profiling of Dystroglycan-Associated Proteins in Wild Type, mdx, and Galgt2 Transgenic Mouse Skeletal Muscle.  JOURNAL OF PROTEOME RESEARCH. Vol. 11, no. 9. (September): 4413-4424.
  • Johnson,Eric,K; Zhang,Liwen; Adams,Marvin,E; Phillips,Alistair; Freitas,Michael,A; Froehner,Stanley,C; Green-Church,Kari,B; Montanaro,Federica. 2012. Proteomic Analysis Reveals New Cardiac-Specific Dystrophin-Associated Proteins.  PLOS ONE. Vol. 7, no. 8. (August): ee43515.
  • Beastrom N., Lu H., Macke A., Canan B.D., Johnson E.K., Penton C.M., Kaspar B.K., Rodino-Klapac L.R., Zhou L., Janssen P.M.L., Montanaro F. 2011. Mdx5cv mice manifest more severe muscle dysfunction and diaphragm force deficits than mdx mice.  American Journal of Pathology. Vol. 179, no. Electronic Publication Ahead of Print. (November): 2464-74.
  • Leblanc E; Trensz F; Haroun S; Drouin G; Bergeron E; Penton CM; Montanaro F; Roux S; Faucheux N; Grenier G. 2011. BMP-9-induced muscle heterotopic ossification requires changes to the skeletal muscle microenvironment.  Journal Of Bone And Mineral Research: The Official Journal Of The American Society For Bone And Mineral Research. Vol. 26, no. 6. (June): e1166.
  • Alexander MS; Kawahara G; Kho AT; Howell MH; Pusack TJ; Myers JA; Montanaro F; Zon LI; Guyon JR; Kunkel LM. 2011. Isolation and transcriptome analysis of adult zebrafish cells enriched for skeletal muscle progenitors.  Muscle & Nerve. Vol. 43, no. 5. (May): e741.
  • Montanaro F, Martin PT. 2011. Defective Glycosylation of Dystroglycan in Muscular Dystrophy and Cancer. In Post-Translational Modifications in Health and Disease. Edited by Cecilio Vidal Moreno.: SPRINGER.
  • Montanaro,Federica; Allen,Hugh,D; Mendell,Jerry,R; Kaspar,Rita,Wen; Flanigan,Kevin,M; Kissel,John,T; Pestronk,Alan; Weiss,Robert,B; Ray,Will; Alvarez,Carlos,E. 2010. Letter by Yilmaz et al Regarding Article, "Analysis of Dystrophin Deletion Mutations Predicts Age of Cardiomyopathy Onset in Becker Muscular Dystrophy" Response.  CIRCULATION-CARDIOVASCULAR GENETICS. Vol. 3, no. 2. (April): E2-E2.
  • Kaspar RW; Allen HD; Ray WC; Alvarez CE; Kissel JT; Pestronk A; Weiss RB; Flanigan KM; Mendell JR; Montanaro F. 2009. Analysis of dystrophin deletion mutations predicts age of cardiomyopathy onset in becker muscular dystrophy.  Circulation: Cardiovascular Genetics. Vol. 2, no. 6. (December): e544.
  • Kaspar RW; Allen HD; Montanaro F. 2009. Current understanding and management of dilated cardiomyopathy in Duchenne and Becker muscular dystrophy.  Journal Of The American Academy Of Nurse Practitioners. Vol. 21, no. 5. (May): e241.
  • Liadaki K, Montanaro F, and Kunkel LM. 2006. Cellular-mediated delivery: the intersection between regenerative medicineand genetic therapy. In Duchenne muscular dystrophy: Advances in therapeutics. First edition ed. Edited by Rando T. and Chamberlain J. New York, NY: Taylor and Francis Group - Informa.
  • Guyon JR; Mosley AN; Jun SJ; Montanaro F; Steffen LS; Zhou Y; Nigro V; Zon LI; Kunkel LM. 2005. Delta-sarcoglycan is required for early zebrafish muscle organization.  Experimental Cell Research. Vol. 304, no. 1. (March): 105-115.
  • Montanaro F; Liadaki K; Schienda J; Flint A; Gussoni E; Kunkel LM. 2004. Demystifying SP cell purification: viability, yield, and phenotype are defined by isolation parameters.  Experimental Cell Research. Vol. 298, no. 1. (August): 144-154.
  • Tomczak KK; Marinescu VD; Ramoni MF; Sanoudou D; Montanaro F; Han M; Kunkel LM; Kohane IS; Beggs AH. 2004. Expression profiling and identification of novel genes involved in myogenic differentiation.  The FASEB Journal: Official Publication Of The Federation Of American Societies For Experimental Biology. Vol. 18, no. 2. (February): 403-405.
  • Tomczak,K,K; Marinescu,V,D; Ramoni,M,F; Sanoudou,D; Montanaro,F; Han,M; KUNKEL,L,M; Kohane,I,S; Beggs,A,H. 2003. Expression profiling and identification of novel genes involved in myogenic differentiation.  FASEB JOURNAL. Vol. 17, no. 15. (December): 403-?.
  • Montanaro F; Liadaki K; Volinski J; Flint A; Kunkel L. 2003. Skeletal muscle engraftment potential of adult mouse skin side population cells.  Proceedings of the National Academy of Sciences of the United States of America. Vol. 100, no. 16. (January): 9336-9341.
  • Montanaro F. and Carbonetto S. 2003. Targeting dystroglycan in the brain.  Neuron. Vol. 37, no. 2. (January): 193-196.
  • Montanaro,F; Carbonetto,S. 2003. Targeting dystroglycan in the brain.  NEURON. Vol. 37, no. 2. (January): 193-196.
  • Montanaro F, Carbonetto S. 2000. Developmental biology of synapse formation.  Encyclopedia of Life Sciences.
  • Montanaro F; Lindenbaum M; Carbonetto S. 1999. alpha-Dystroglycan is a laminin receptor involved in extracellular matrix assembly on myotubes and muscle cell viability.  The Journal Of Cell Biology. Vol. 145, no. 6. (June): 1325-1340.
  • Jacobson C; Montanaro F; Lindenbaum M; Carbonetto S; Ferns M. 1998. alpha-Dystroglycan functions in acetylcholine receptor aggregation but is not a coreceptor for agrin-MuSK signaling.  The Journal Of Neuroscience: The Official Journal Of The Society For Neuroscience. Vol. 18, no. 16. (August): 6340-6348.
  • Montanaro F; Gee SH; Jacobson C; Lindenbaum MH; Froehner SC; Carbonetto S. 1998. Laminin and alpha-dystroglycan mediate acetylcholine receptor aggregation via a MuSK-independent pathway.  The Journal Of Neuroscience: The Official Journal Of The Society For Neuroscience. Vol. 18, no. 4. (February): 1250-1260.
  • Montanaro F; Carbonetto S; Campbell KP; Lindenbaum M. 1995. Dystroglycan expression in the wild type and mdx mouse neural retina: synaptic colocalization with dystrophin, dystrophin-related protein but not laminin.  Journal Of Neuroscience Research. Vol. 42, no. 4. (November): 528-538.
  • Gee SH; Montanaro F; Lindenbaum MH; Carbonetto S. 1994. Dystroglycan-alpha, a dystrophin-associated glycoprotein, is a functional agrin receptor.  Cell. Vol. 77, no. 5. (June): e675.
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700 Children's Drive Columbus, Ohio 43205 614.722.2000