Mice with pR927Q and pI712M <i>Erbb4</i> Mutations Develop Features of Frontotemporal Dementia and ALS

Initial findings suggest gene dosage-dependent abnormalities in Erbb4-R927Q mutants that have features of FTLD and ALS. A wider battery of tests on more animals, at different ages and including the Erbb4-I712M mutants are underway. Neuropathology is being assessed following consensus recommendations for FTLD and ALS. Stereology/morphometry and other methods will assess loss of ErbB4-expressing inhibitory interneurons and synaptic loss and how this evolves temporally.