Jordi Magrane, Associate Professor of Research in Neuroscience
Name: B6.Cg-Tg(Nes-cre)1Kln Fxnem2Lutzy Fxnem8Lutzy
Modification: Transgenic
Description: Nervous system-specific frataxin (FXN) depletion mouse model of Friedreich’s Ataxia (FRDA). This mouse presents a FXN G127V missense mutation inserted into one allele of the mouse Fxn gene, a Cre-conditional frataxin allele (exon 2 floxed), and the rat nestin promoter and enhancer controlling the expression of Cre recombinase (which drives its expression in the central and peripheral nervous systems). Mice are characterized by severe depletion of FXN in both glia and neurons in the nervous system from neonatal stages to adulthood. Some residual FXN expressed, as in humans with FRDA. Mice demonstrate progressive neurobehavioral deficits that appear around 1.5-2.5 months of age.
Application: Mouse model useful for studying Friedreich’s Ataxia disease biology and therapeutic strategies targeted to the nervous system.
Availability: Available from WCM.
*Availability and licensing conditions are subject to change.
Intellectual Property
Cornell Reference
- D-11214
Contact Information
For additional information please contact
Ivan Gando
Business Development and Licensing Associate
Phone: (646) 814-6459
Email: ivan.gando@cornell.edu