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Hekmatara, M.

Publications and source records attributed to Hekmatara, M..

2 recordsLinked to original sources

Super-resolution microscopy reveals a Rab6a-dependent trafficking hub for rhodopsin at the mammalian rod photoreceptor Golgi

Rod photoreceptor stability is critical for retinal health and lifelong vision. The proper intracellular trafficking of the photopigment receptor rhodopsin (Rho) is essential for normal rod homeostasis, as Rho mislocalization precedes rod cell death in inherited retinal disorders such as retinitis pigmentosa. Despite its importance, the molecular mechanisms of Rho trafficking in mammalian rods remain largely undefined. In this study, we combined multiple Rho-labeling strategies with super-resolution microscopy to investigate the subcellular organization of Rho in the Golgi complex of mammalian rods. Using stochastic optical reconstruction microscopy (STORM) and structured illumination microscopy (SIM) super-resolution imaging modalities, we mapped the localization of Golgi proteins with Rho in mouse and macaque rods and found that Rho specifically colocalizes with Rab6a in the trans-Golgi. To test the functional significance of this interaction, we utilized a dominant-negative Rab6a mutant in both HEK293T cells and mouse rods. We demonstrated that the dominant negative Rab6a significantly inhibits Rho secretion in cell culture, causing intracellular retention. In mouse rods, we found that this mutant similarly causes significant Rho retention in the trans-Golgi. However, surprisingly, a majority of Rho protein still escaped the Golgi and reached the outer segment. Together, these findings uncover critical new subcellular details about Rho organization at the Golgi and establish a role for Rab6a as a regulator of Rho protein release from the trans-Golgi in mammalian rods. Our results provide critical insight into the protein trafficking mechanisms that must be sustained and regulated in mammalian rods for long-term retinal health.

cell biology↗

ER Aggregation Causes Synaptic Protein Imbalance in Retinitis Pigmentosa Mutant Photoreceptor Neurons

Rod photoreceptor neurons in the retina detect scotopic light through the visual pigment rhodopsin (Rho) in their outer segments (OS). Efficient Rho trafficking to the OS through the inner rod compartments is critical for long-term rod health. Given the importance of protein trafficking to the OS, less is known about the trafficking of rod synaptic proteins. Furthermore, the subcellular impact of Rho mislocalization on rod synapses (i.e., "spherules") has not been investigated. In this study we used super-resolution and electron microscopies, along with proteomics, to perform a subcellular analysis of Rho synaptic mislocalization in P23H-Rho-RFP mutant mice. We discovered that mutant P23H-Rho-RFP protein mislocalized in distinct ER aggregations within the spherule cytoplasm, which we confirmed with AAV overexpression. Additionally, we found synaptic protein abundance differences in P23H-Rho-RFP mice. By comparison, Rho mislocalized along the spherule plasma membrane in WT and rd10 mutant rods, in which there was no synaptic protein disruption. Throughout the study, we also identified a network of ER membranes within WT rod presynaptic spherules. Together, our findings indicate that photoreceptor synaptic proteins are sensitive to ER dysregulation. Summary StatementThis study examines the impact of rhodopsin mislocalization on rod photoreceptor synaptic structures and synaptic protein levels using P23H rhodopsin and other retinitis pigmentosa mouse models.

neuroscience↗