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  • Rescuing Z+ agrin splicing in Nova null mice restores synapse formation and unmasks a physiologic defect in motor neuron firing
    Ruggiu, Matteo ...
    Synapse formation at the neuromuscular junction (NMJ) requires an alternatively spliced variant of agrin (Z(+) agrin) that is produced only by neurons. Here, we show that Nova1 and Nova2, ... neuron-specific splicing factorsidentified as targets in autoimmune motor disease, are essential regulators of Z(+) agrin. Nova1/Nova2 double knockout mice are paralyzed and fail to cluster AChRs at the NMJ, and breeding them with transgenic mice constitutively expressing Z(+) agrin in motor neurons rescued AChR clustering. Surprisingly, however, these rescued mice remained paralyzed, while electrophysiologic studies demonstrated that the motor axon and synapsewere functional-spontaneous and evoked recordings revealed synaptic transmission and muscle contraction. These results point to a proximal defect in motor neuron firing in the absence of Nova and reveal a previously unsuspected role for RNA regulation in the physiologic activation of motor neurons.
    Vir: Proceedings of the National Academy of Sciences of the United States of America. - ISSN 0027-8424 (Letn. 106, št. 9, 2009, str. 3513-3518)
    Vrsta gradiva - članek, sestavni del
    Leto - 2009
    Jezik - angleški
    COBISS.SI-ID - 25482201
    DOI

Vnos na polico