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The role of Purkinje cell-derived VEGF in cerebellar astrogliosis in Niemann-Pick type C mice
Hee Kyung Jin1,4,*,#, Min Hee Park1,2,3, Ju Youn Lee1,2,3, Min Seock Jeong1,4, Hyung Sup Jang1,4, Shogo Endo5, Jae-sung Bae1,2,3,#
1Stem Cell Neuroplasticity Research Group, Kyungpook National University,
2Department of Physiology, School of Medicine, Kyungpook National University,
3Department of Biomedical Science, BK21 Plus KNU Biomedical Convergence Progra and 4College of Veterinary Medicine, Kyungpook National University,
5Aging Neuroscience Research Team, Tokyo Metropolitan Institute of Gerontology
Abstract
Niemann-Pick type C disease (NP-C) is a fatal neurodegenerative disorder caused by a deficiency of NPC1 gene function, which leads to severe neuroinflammation such as astrogliosis. While reports demonstrating neuroinflammation are prevalent in NP-C, information about the onset and progression of cerebellar astrogliosis in this disorder is lacking. Using gene targeting, we generated vascular endothelial growth factor (VEGF) conditional null mutant mice. Deletion of VEGF in cerebellar Purkinje neurons (PNs) led to a significant increase of astrogliosis in the brain of NP-C mice in addition to the loss of PNs, suggesting PN-derived VEGF as an important factor in NP-C pathology. Moreover, replenishment of VEGF in neurons improved brain pathology in NP-C mice. Overall, our data provide a new pathological perspective on cerebellar astrogliosis in NP-C and suggest the importance of VEGF as a therapeutic target for this disease.
Abstract, Accepted Manuscript(in press) [Submitted on August 26, 2017, Accepted on October 20, 2017]
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