Pikachurin, a dystroglycan ligand, is essential for photoreceptor ribbon synapse formation
doi: 10.1038/nn.2160
pmid: 18641643
Pikachurin, a dystroglycan ligand, is essential for photoreceptor ribbon synapse formation
Exquisitely precise synapse formation is crucial for the mammalian CNS to function correctly. Retinal photoreceptors transfer information to bipolar and horizontal cells at a specialized synapse, the ribbon synapse. We identified pikachurin, an extracellular matrix-like retinal protein, and observed that it localized to the synaptic cleft in the photoreceptor ribbon synapse. Pikachurin null-mutant mice showed improper apposition of the bipolar cell dendritic tips to the photoreceptor ribbon synapses, resulting in alterations in synaptic signal transmission and visual function. Pikachurin colocalized with both dystrophin and dystroglycan at the ribbon synapses. Furthermore, we observed direct biochemical interactions between pikachurin and dystroglycan. Together, our results identify pikachurin as a dystroglycan-interacting protein and demonstrate that it has an essential role in the precise interactions between the photoreceptor ribbon synapse and the bipolar dendrites. This may also advance our understanding of the molecular mechanisms underlying the retinal electrophysiological abnormalities observed in muscular dystrophy patients.
- Osaka Bioscience Institute Japan
- Osaka Gakuin University Japan
- Osaka University Japan
- Nagoya University Japan
- Tokai National Higher Education and Research System Japan
Extracellular Matrix Proteins, Molecular Sequence Data, Mice, Transgenic, Blotting, Northern, Ligands, Macaca mulatta, Mice, Mutant Strains, Extracellular Matrix, Dystrophin, Mice, Organ Specificity, Animals, Humans, Cattle, Photoreceptor Cells, Amino Acid Sequence, Dystroglycans, Eye Proteins, Chickens, In Situ Hybridization
Extracellular Matrix Proteins, Molecular Sequence Data, Mice, Transgenic, Blotting, Northern, Ligands, Macaca mulatta, Mice, Mutant Strains, Extracellular Matrix, Dystrophin, Mice, Organ Specificity, Animals, Humans, Cattle, Photoreceptor Cells, Amino Acid Sequence, Dystroglycans, Eye Proteins, Chickens, In Situ Hybridization
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