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1 Neurology, Baylor College of Medicine, Houston, TX, USA; Neuroscience, Baylor College of Medicine, USA
2 Neurology, Baylor College of Medicine, Houston, TX, USA
3 Cell Biology and Histology, University of Nijmegen, Nijmegen, Netherlands Antilles
4 Neurology, Baylor College of Medicine, Houston, TX, USA; Neuroscience, Baylor College of Medicine, USA; Biochemistry and Molecular Biology, Baylor College of Medicine, USA
* To whom correspondence should be addressed. E-mail: pschulz{at}bcm.tmc.edu.
Myotonic dystrophy (DM) is associated with an expanded triplet repeat in the 3'-untranslated region of the gene for myotonic dystrophy protein kinase (DMPK), which may reduce DMPK expression. It is unclear how reduced DMPK expression might contribute to the symptoms of DM because the normal function of DMPK is not yet understood. Thus, we investigated the function of DMPK in order to gain insight into how reduced DMPK expression might lead to cognitive dysfunction in DM. We recently demonstrated a role for DMPK in modifying the cytoskeleton, and remodeling of the cytoskeleton is thought to be important for cognitive function. Therefore, we hypothesized that DMPK might normally contribute to synaptic plasticity and cognitive function via an effect on actin cytoskeletal rearrangements. To test for involvement of DMPK in synaptic plasticity, we utilized the DMPK null mouse. This mouse showed no changes in baseline synaptic transmission in hippocampal area CA1, nor any changes in long-term synaptic potentiation (LTP) measured three hours after induction. There was a significant decrease, however, in the decremental potentiation with a duration of 30-180 minutes that accompanies LTP. These results suggest a role for DMPK in synaptic plasticity that could be relevant to the cognitive dysfunction associated with DM.
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