Detail

Keywords

Arrhythmias, Cardiac; Calcium Channels, L-Type; Cardiac Electrophysiology; Cardiomyopathy, Dilated; Muscular Dystrophy, Duchenne; Voltage-Gated Sodium Channels

Research group(s)

• Muscle physiology
  Head: Karlheinz Hilber
  Research Area: Skeletal and cardiac muscle physiology
  Members:
  Xaver König
  Karlheinz Hilber

Research interests

• Cardiac ion channel impairments in the diseased heart
• Effects of drugs on cardiac ion channels

Techniques, methods & infrastructure

whole cell patch clamp technique, isolation of cardiomyocytes from neonatal and adult mouse and guinea pig hearts, human induced pluripotent stem cell- derived cardiomyocytes, muscular dystrophy mouse models, standard biochemistry and molecular biology 

Members of this research group have access to a cell culture, a standard biochemistry and molecular biology lab, and a patch clamp rig.

Grants

• Ion channels in dystrophic cardiac Purkinje cells (2022)
  Source of Funding: FWF (Austrian Science Fund), Stand-alone project
  Principal Investigator
• Role of tenascin C in electrical remodeling and impaired Ca handling in dystrophic cardiomyopathy (2021)
  Source of Funding: FWF (Austrian Science Fund), Stand-alone project
  Principal Investigator
• Ivabradine rescue of dystrophic cardiomyopathy using small animal models for Duchenne muscular dystrophy (2020)
  Source of Funding: Österreichische Muskelforschung, Stand alone project
  Principal Investigator
• Cardiac calcium channel abnormalities in Duchenne Muscular Dystrophy (2017)
  Source of Funding: FWF (Austrian Science Fund), Stand-Alone Projects
  Principal Investigator
• Voltage-gated ion channel dysfunction in dystrophic muscle (2010)
  Source of Funding: FWF (Austrian Science Fund), Stand-Alone Projects
  Principal Investigator
• Cardiogenic substances (2009)
  Source of Funding: aws (austria wirtschaftsservice), PRIZE
  Principal Investigator

Selected publications

1. Ebner, J. et al. (2022) ‘Microdystrophin Therapy Rescues Impaired Na Currents in Cardiac Purkinje Fibers From Dystrophin-Deficient Mdx Mice’, Circulation: Arrhythmia and Electrophysiology, 15(8). Available at: http://dx.doi.org/10.1161/circep.122.011161.
2. Ebner, J. et al. (2020) ‘Reduced Na+ current in Purkinje fibers explains cardiac conduction defects and arrhythmias in Duchenne muscular dystrophy’, American Journal of Physiology-Heart and Circulatory Physiology, 318(6), pp. H1436–H1440. Available at: http://dx.doi.org/10.1152/ajpheart.00224.2020.
3. Koenig, X., Ebner, J. and Hilber, K. (2018) ‘Voltage-Dependent Sarcolemmal Ion Channel Abnormalities in the Dystrophin-Deficient Heart’, International Journal of Molecular Sciences, 19(11), p. 3296. Available at: http://dx.doi.org/10.3390/ijms19113296.
4. Koenig, X. et al. (2014) ‘Enhanced currents through L-type calcium channels in cardiomyocytes disturb the electrophysiology of the dystrophic heart’, American Journal of Physiology-Heart and Circulatory Physiology, 306(4), pp. H564–H573. Available at: http://dx.doi.org/10.1152/ajpheart.00441.2013.
5. Koenig, X. et al. (2012) ‘Anti-addiction drug ibogaine inhibits hERG channels: a cardiac arrhythmia risk’, Addiction Biology, 19(2), pp. 237–239. Available at: http://dx.doi.org/10.1111/j.1369-1600.2012.00447.x.