ZFIN ID: ZDB-LAB-180517-2
Lobo Lab
PI/Director: Lobo, Glenn
Contact Person: Lobo, Glenn
Email: lobo@musc.edu
URL: http://academicdepartments.musc.edu/facultydirectory/Lobo-Glenn
Address: Medical University of South Carolina Department of Medicine Drug Discovery Building Room: DDB520 70 President Street, MSC 629 Charleston, SC 29425
Country: United States
Phone: 843-876-2371
Line Designation: muz

Show all 4 genomic features

I am a basic scientist interested in studying molecular mechanisms of retinal cell degeneration and regeneration. Following PhD training in molecular and cellular biology, I pursued fellowships in the molecular genetics of human retinal degenerations and in the study of receptor mediated Vitamin A transport for vision. As a Postdoctoral Research Associate in Dr. Kris Palczewski and Dr. Johannes von Lintig Laboratories (Department of Pharmacology, Case Western Reserve University), I identified functional roles for membrane receptors in the uptake (SR-B1), metabolism (BCMO1 and BCMO2) and transport (RBPR2 and STRA6) of Vitamin A/ Retinol for vision. During this period I gained a broad background and experience in molecular genetics and cellular biology of vision. With this experience, I then focused on identifying the mechanisms of mutant TULP1 in causing photoreceptor degeneration in Retinitis Pigmentosa (RP) and Leber congenital amaurosis (LCA) patients. Here, as a Project Scientist in Dr. Stephanie Hagstrom Laboratory (Cole Eye Institute, Cleveland Clinic), I identified that mutant misfolded TULP1 protein, accumulate within the ER and cause induction of the unfolded protein response complex, leading to apoptosis, thereby identifying for the first time the molecular pathway of action and the pathologic mechanism responsible for the retinal degeneration caused by TULP1 mutations. My current research projects aim to investigate role of novel receptors (STRA6l/ RBPR2) and mechanisms thereof for systemic uptake of vitamin A from its protein bound form (RBP4-ROL) and peripheral tissue storage of vitamin A for chromophore production in sustaining human vision. In line with this, my current research specifically focuses on understanding mechanisms of retinal degeneration and injury associated with low systemic Vitamin A levels using mutant zebrafish and mouse models devoid of the RBPR2 vitamin A transporter. In collaboration with Dr. Barbel Rohrer and Dr. Joshua Lipschutz (both MUSC) I am also investigating the role of the Exocyst complex in ciliogenesis and mechanisms associated with pathologies of the eye. I apply state-of-the art genomic, biochemical and molecular biology technologies, including CRISPR-generated mutant and knock-in zebrafish lines lacking novel receptors for Vitamin A uptake, previously established mouse models of vitamin A deficiency, to determine the physiological consequences of low vitamin A and its effects on vertebrate RPE and photoreceptor health. These studies will help elaborate potential underlying mechanisms responsible for linkages between systemic Vitamin A uptake and storage and chronic visual diseases and help develop novel strategies in maintaining human visual functions.

Shi, Yi Post-Doc Su, Yanhui Research Staff

Solanki, A.K., Kondkar, A.A., Fogerty, J., Su, Y., Kim, S.H., Lipschutz, J.H., Nihalani, D., Perkins, B.D., Lobo, G.P. (2020) A Functional Binding Domain in the Rbpr2 Receptor Is Required for Vitamin A Transport, Ocular Retinoid Homeostasis, and Photoreceptor Cell Survival in Zebrafish. Cells. 9(5):
Fulmer, D., Toomer, K., Guo, L., Moore, K., Glover, J., Moore, R., Stairley, R., Lobo, G., Zuo, X., Dang, Y., Su, Y., Fogelgren, B., Gerard, P., Chung, D., Heydarpour, M., Mukherjee, R., Body, S.C., Norris, R.A., Lipschutz, J.H. (2019) Defects in the Exocyst-Cilia Machinery Cause Bicuspid Aortic Valve Disease and Aortic Stenosis. Circulation. 140(16):1331-1341
Wang, W., Fang, D.H., Gan, J., Shi, Y., Tang, H., Wang, H., Fu, M.Z., Yi, J. (2019) Evolutionary and functional implications of 3' untranslated region length of mRNAs by comprehensive investigation among four taxonomically diverse metazoan species. Genes & genomics. 41(7):747-755
Zuo, X., Lobo, G., Fulmer, D., Guo, L., Dang, Y., Su, Y., Ilatovskaya, D.V., Nihalani, D., Rohrer, B., Body, S.C., Norris, R.A., Lipschutz, J.H. (2019) The exocyst acting through the primary cilium is necessary for renal ciliogenesis, cystogenesis, and tubulogenesis. The Journal of biological chemistry. 294(17):6710-6718
Lobo, G.P., Pauer, G., Lipschutz, J.H., Hagstrom, S.A. (2018) The Retinol-Binding Protein Receptor 2 (Rbpr2) Is Required for Photoreceptor Survival and Visual Function in the Zebrafish. Advances in experimental medicine and biology. 1074:569-576
Shi, Y., Su, Y., Lipschutz, J.H., Lobo, G.P. (2017) Zebrafish as models to study ciliopathies of the eye and kidney. Clinical nephrology and research. 1:6-9
Shi, Y., Obert, E., Rahman, B., Rohrer, B., Lobo, G.P. (2017) The Retinol Binding Protein Receptor 2 (Rbpr2) is required for Photoreceptor Outer Segment Morphogenesis and Visual Function in Zebrafish. Scientific Reports. 7:16207
Lobo, G.P., Fulmer, D., Guo, L., Zuo, X., Dang, Y., Kim, S.H., Su, Y., George, K., Obert, E., Fogelgren, B., Nihalani, D., Norris, R.A., Rohrer, B., Lipschutz, J.H. (2017) The exocyst is required for photoreceptor ciliogenesis and retinal development. The Journal of biological chemistry. 292(36):14814-14826
Daniele, L.L., Emran, F., Lobo, G.P., Gaivin, R.J., Perkins, B.D. (2016) Mutation of wrb, a Component of the Guided Entry of Tail-Anchored Protein Pathway, Disrupts Photoreceptor Synapse Structure and Function. Investigative ophthalmology & visual science. 57:2942-54
Kim, S.H., Wu, S.Y., Baek, J.I., Choi, S.Y., Su, Y., Flynn, C.R., Gamse, J.T., Ess, K.C., Hardiman, G., Lipschutz, J.H., Abumrad, N.N., Rockey, D.C. (2015) A Post-Developmental Genetic Screen for Zebrafish Models of Inherited Liver Disease. PLoS One. 10:e0125980
Lobo, G.P., Isken, A., Hoff, S., Babino, D., and von Lintig, J. (2012) BCDO2 acts as a carotenoid scavenger and gatekeeper for the mitochondrial apoptotic pathway. Development (Cambridge, England). 139(16):2966-2977