Methods and transgenic mouse model for identifying and modulating factors leading to motor neuron degeneration

摘要

Methods are provided for identifying factors involved in motor neuron degeneration by identifying factors affected by alterations in a C-binding complex of a ribonucleotide protein complex, isolating and purifying the factors or nucleic acid sequences; and examining the neuropathic effect of the factors or nucleic acid sequences encoding the factors in a transgenic mouse. Also provided is a transgenic mouse model which expresses a mutant transgenic NF mRNA and exhibits degeneration of motor neurons. This model can be used to test, screen and identify factors and nucleic acid sequences encoding factors causing degeneration of motor neurons.