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Progranulin

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https://www.readbyqxmd.com/read/28509387/progranulin-increases-phagocytosis-by-retinal-pigment-epithelial-cells-in-culture
#1
Hiromi Murase, Kazuhiro Tsuruma, Yoshiki Kuse, Masamitsu Shimazawa, Hideaki Hara
Retinal pigment epithelium (RPE) cells take part in retinal preservation, such as phagocytizing the shed photoreceptor outer segments (POS), every day. The incomplete phagocytic function accelerates RPE degeneration and formation of the toxic by-product lipofuscin. Excessive lipofuscin accumulation is characteristic of various blinding diseases in the human eye. Progranulin is a cysteine-rich protein that has multiple biological activities, and it has a high presence in the retina. Progranulin has been recognized to be involved in macrophage phagocytosis in the brain...
May 16, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28490764/progranulin-deficiency-causes-the-retinal-ganglion-cell-loss-during-development
#2
Yoshiki Kuse, Kazuhiro Tsuruma, Takahiro Mizoguchi, Masamitsu Shimazawa, Hideaki Hara
Astrocytes are glial cells that support and protect neurons in the central nervous systems including the retina. Retinal ganglion cells (RGCs) are in contact with the astrocytes and our earlier findings showed the reduction of the number of cells in the ganglion cell layer in adult progranulin deficient mice. In the present study, we focused on the time of activation of the astrocytes and the alterations in the number of RGCs in the retina and optic nerve in progranulin deficient mice. Our findings showed that the number of Brn3a-positive cells was reduced and the expression of glial fibrillary acidic protein (GFAP) was increased in progranulin deficient mice...
May 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28479389/involvement-of-progranulin-in-modulating-neuroinflammatory-responses-but-not-neurogenesis-in-the-hippocampus-of-aged-mice
#3
Yanbo Ma, Takashi Matsuwaki, Keitaro Yamanouchi, Masugi Nishihara
It is well established that adult neurogenesis in the hippocampus declines with age. Our previous studies have suggested that progranulin (PGRN) has a facilitative effect on hippocampal neurogenesis. We have also shown that PGRN plays a role in suppressing excessive neuroinflammatory responses in the cortex and thalamus after brain injury and aging, respectively. However, the roles of PGRN in modulating neurogenesis and neuroinflammatory responses in the hippocampus of aged animals are not yet understood. In the present study, we investigated neurogenesis and neuroinflammation-related responses in the hippocampus of young (15-week-old) and old (135-week-old) wild-type and PGRN-deficient male mice...
May 4, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28473694/accumulation-of-multiple-neurodegenerative-disease-related-proteins-in-familial-frontotemporal-lobar-degeneration-associated-with-granulin-mutation
#4
Masato Hosokawa, Hiromi Kondo, Geidy E Serrano, Thomas G Beach, Andrew C Robinson, David M Mann, Haruhiko Akiyama, Masato Hasegawa, Tetsuaki Arai
In 2006, mutations in the granulin gene were identified in patients with familial Frontotemporal Lobar Degeneration. Granulin transcript haploinsufficiency has been proposed as a disease mechanism that leads to the loss of functional progranulin protein. Granulin mutations were initially found in tau-negative patients, though recent findings indicate that these mutations are associated with other neurodegenerative disorders with tau pathology, including Alzheimer's disease and corticobasal degeneration. Moreover, a reduction in progranulin in tau transgenic mice is associated with increasing tau accumulation...
May 4, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28472860/peripheral-nerve-atrophy-together-with-higher-csf-progranulin-indicate-axonal-damage-in-als
#5
Stefanie Schreiber, Grazyna Debska-Vielhaber, Susanne Abdulla, Judith Machts, Frank Schreiber, Siegfried Kropf, Peter Körtvelyessy, Sonja Körner, Katja Kollewe, Susanne Petri, Reinhard Dengler, Wolfram S Kunz, Peter J Nestor, Stefan Vielhaber
INTRODUCTION: We aimed to investigate whether sonographic peripheral cross-sectional nerve area (CSA) and progranulin (PGRN), a neuritic growth factor, are related to each other and whether they interact to predict clinical and paraclinical measures in amyotrophic lateral sclerosis (ALS). METHODS: We included 55 ALS patients who had forearm median and ulnar nerve CSA, cerebrospinal fluid (CSF) PGRN and serum PGRN measures available. CSF PGRN was normalized against the CSF/serum albumin ratio (Qalb )...
May 4, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28462717/genetic-features-of-mapt-grn-c9orf72-and-chchd10-gene-mutations-in-chinese-patients-with-frontotemporal-dementia
#6
Xiang-Qian Che, Qian-Hua Zhao, Yue Huang, Xia Li, Ru-Jing Ren, Sheng-Di Chen, Gang Wang, Qi-Hao Guo
Mutations in microtubule associated protein tau (MAPT), progranulin (GRN), chromosome 9 open-reading frame 72 (C9orf72) and CHCHD10 genes have been reported causing frontotemporal dementia (FTD) in different populations. However, collective analysis of mutations in these four genes in Chinese FTD patients has not been reported yet. The aim of this study was to investigate the geneticfeatures of Chinese patients with MAPT, PGRN, C9orf72 or CHCHD10 gene mutations in a FTD cohort recruited from multi clinical centers in Shanghai metropolitan areas, China...
April 25, 2017: Current Alzheimer Research
https://www.readbyqxmd.com/read/28453791/progranulin-functions-as-a-cathepsin-d-chaperone-to-stimulate-axonal-outgrowth-in-vivo
#7
Sander Beel, Matthieu Moisse, Markus Damme, Louis De Muynck, Wim Robberecht, Ludo Van Den Bosch, Paul Saftig, Philip Van Damme
Loss of function mutations in progranulin (GRN) cause frontotemporal dementia, but how GRN haploinsufficiency causes neuronal dysfunction remains unclear. We previously showed that GRN is neurotrophic in vitro. Here, we used an in vivo axonal outgrowth system and observed a delayed recovery in GRN-/- mice after facial nerve injury. This deficit was rescued by reintroduction of human GRN and relied on its C-terminus and on neuronal GRN production. Transcriptome analysis of the facial motor nucleus post injury identified cathepsin D (CTSD) as the most upregulated gene...
April 26, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28438992/microglial-nf%C3%AE%C2%BAb-tnf%C3%AE-hyperactivation-induces-obsessive-compulsive-behavior-in-mouse-models-of-progranulin-deficient-frontotemporal-dementia
#8
Grietje Krabbe, S Sakura Minami, Jon I Etchegaray, Praveen Taneja, Biljana Djukic, Dimitrios Davalos, David Le, Iris Lo, Lihong Zhan, Meredith C Reichert, Faten Sayed, Mario Merlini, Michael E Ward, David C Perry, Suzee E Lee, Ana Sias, Christopher N Parkhurst, Wen-Biao Gan, Katerina Akassoglou, Bruce L Miller, Robert V Farese, Li Gan
Frontotemporal dementia (FTD) is the second most common dementia before 65 years of age. Haploinsufficiency in the progranulin (GRN) gene accounts for 10% of all cases of familial FTD. GRN mutation carriers have an increased risk of autoimmune disorders, accompanied by elevated levels of tissue necrosis factor (TNF) α. We examined behavioral alterations related to obsessive-compulsive disorder (OCD) and the role of TNFα and related signaling pathways in FTD patients with GRN mutations and in mice lacking progranulin (PGRN)...
April 24, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28435163/progranulin-lysosomal-regulation-and-neurodegenerative-disease
#9
REVIEW
Aimee W Kao, Andrew McKay, Param Priya Singh, Anne Brunet, Eric J Huang
The discovery that heterozygous and homozygous mutations in the gene encoding progranulin are causally linked to frontotemporal dementia and lysosomal storage disease, respectively, reveals previously unrecognized roles of the progranulin protein in regulating lysosome biogenesis and function. Given the importance of lysosomes in cellular homeostasis, it is not surprising that progranulin deficiency has pleiotropic effects on neural circuit development and maintenance, stress response, innate immunity and ageing...
June 2017: Nature Reviews. Neuroscience
https://www.readbyqxmd.com/read/28433812/the-perlecan-interacting-growth-factor-progranulin-regulates-ubiquitination-sorting-and-lysosomal-degradation-of-sortilin
#10
Ryuta Tanimoto, Chiara Palladino, Shi-Qiong Xu, Simone Buraschi, Thomas Neill, Leonard G Gomella, Stephen C Peiper, Antonino Belfiore, Renato V Iozzo, Andrea Morrione
Despite extensive clinical and experimental studies over the past decades, the pathogenesis and progression to the castration-resistant stage of prostate cancer remains largely unknown. Progranulin, a secreted growth factor, strongly binds the heparin-sulfate proteoglycan perlecan, and counteracts its biological activity. We established that progranulin acts as an autocrine growth factor and promotes prostate cancer cell motility, invasion, and anchorage-independent growth. Progranulin was overexpressed in prostate cancer tissues vis-à-vis non-neoplastic tissues supporting the hypothesis that progranulin may play a key role in prostate cancer progression...
April 20, 2017: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/28415822/interaction-between-granulin-a-and-enolase-1-attenuates-the-migration-and-invasion-of-human-hepatoma-cells
#11
Xiaoliang Chen, Huanli Xu, Ning Wu, Xiujun Liu, Gan Qiao, Shuonan Su, Ye Tian, Ru Yuan, Cong Li, Xiaohui Liu, Xiukun Lin
Granulin A (GRN A), a peptide with a molecular 6 kDa, is derived from proteolysis of progranulin (PGRN). Previous study in our laboratory has shown that GRN A is able to inhibit cancer cell growth significantly. In the present study, we confirmed that GRN A can bind to α-enolase (ENO1) specifically as analyzed using Pull-down/MS approaches. The interaction of GRN A with ENO1 was further confirmed by Western blotting and Surface plasmon resonance (SPR) analysis. Treatment of human HepG-2 cells with GRN A inhibited cancer cell growth as well as migration and invasion of cancer cells as analyzed by the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl tetrazoliumbromide (MTT) and Scratch wound healing assay as well as Transwell experiments...
May 2, 2017: Oncotarget
https://www.readbyqxmd.com/read/28412748/tumor-necrosis-factor-receptor-2-akt-and-erk-signaling-pathways-contribute-to-the-switch-from-fibroblasts-to-cafs-by-progranulin-in-microenvironment-of-colorectal-cancer
#12
Linlin Wang, Dong Yang, Jing Tian, Aiqin Gao, Yihang Shen, Xia Ren, Xia Li, Guosheng Jiang, Taotao Dong
Cancer associated fibroblasts (CAFs) are a crucial cellular component in tumor microenvironment and could promote tumor progression. CAFs are usually derived from resident fibroblasts, which undergoing an activated process stimulated by tumor cells. However, the agents and mechanism driving this switch have not yet been elucidated. Progranulin (PGRN), a well acknowledged secreted glycoprotein, could promote proliferation and angiogenesis of colorectal cancer (CRC) cells, and high expression of PGRN correlated with patient poor prognosis...
April 18, 2017: Oncotarget
https://www.readbyqxmd.com/read/28404863/individuals-with-progranulin-haploinsufficiency-exhibit-features-of-neuronal-ceroid-lipofuscinosis
#13
Michael E Ward, Robert Chen, Hsin-Yi Huang, Connor Ludwig, Maria Telpoukhovskaia, Ali Taubes, Helene Boudin, Sakura S Minami, Meredith Reichert, Philipp Albrecht, Jeffrey M Gelfand, Andres Cruz-Herranz, Christian Cordano, Marcel V Alavi, Shannon Leslie, William W Seeley, Bruce L Miller, Eileen Bigio, Marek-Marsel Mesulam, Matthew S Bogyo, Ian R Mackenzie, John F Staropoli, Susan L Cotman, Eric J Huang, Li Gan, Ari J Green
Heterozygous mutations in the GRN gene lead to progranulin (PGRN) haploinsufficiency and cause frontotemporal dementia (FTD), a neurodegenerative syndrome of older adults. Homozygous GRN mutations, on the other hand, lead to complete PGRN loss and cause neuronal ceroid lipofuscinosis (NCL), a lysosomal storage disease usually seen in children. Given that the predominant clinical and pathological features of FTD and NCL are distinct, it is controversial whether the disease mechanisms associated with complete and partial PGRN loss are similar or distinct...
April 12, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28380680/progranulin-regulates-neurogenesis-in-the-developing-vertebrate-retina
#14
Caroline E Walsh, Peter F Hitchcock
We evaluated the expression and function of the microglia-specific growth factor, Progranulin-a (Pgrn-a) during developmental neurogenesis in the embryonic retina of zebrafish. At 24 hpf pgrn-a is expressed throughout the forebrain, but by 48 hpf pgrn-a is exclusively expressed by microglia and/or microglial precursors within the brain and retina. Knockdown of Pgrn-a does not alter the onset of neurogenic programs or increase cell death, however, in its absence, neurogenesis is significantly delayed - retinal progenitors fail to exit the cell cycle at the appropriate developmental time and postmitotic cells do not acquire markers of terminal differentiation, and microglial precursors do not colonize the retina...
April 5, 2017: Developmental Neurobiology
https://www.readbyqxmd.com/read/28379303/restoring-neuronal-progranulin-reverses-deficits-in-a-mouse-model-of-frontotemporal-dementia
#15
Andrew E Arrant, Anthony J Filiano, Daniel E Unger, Allen H Young, Erik D Roberson
Loss-of-function mutations in progranulin (GRN), a secreted glycoprotein expressed by neurons and microglia, are a common autosomal dominant cause of frontotemporal dementia, a neurodegenerative disease commonly characterized by disrupted social and emotional behaviour. GRN mutations are thought to cause frontotemporal dementia through progranulin haploinsufficiency, therefore, boosting progranulin expression from the intact allele is a rational treatment strategy. However, this approach has not been tested in an animal model of frontotemporal dementia and it is unclear if boosting progranulin could correct pre-existing deficits...
March 29, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28358904/neurotrophic-effects-of-progranulin-in-vivo-in-reversing-motor-neuron-defects-caused-by-over-or-under-expression-of-tdp-43-or-fus
#16
Babykumari P Chitramuthu, Denis G Kay, Andrew Bateman, Hugh P J Bennett
Progranulin (PGRN) is a glycoprotein with multiple roles in normal and disease states. Mutations within the GRN gene cause frontotemporal lobar degeneration (FTLD). The affected neurons display distinctive TAR DNA binding protein 43 (TDP-43) inclusions. How partial loss of PGRN causes TDP-43 neuropathology is poorly understood. TDP-43 inclusions are also found in affected neurons of patients with other neurodegenerative diseases including amyotrophic lateral sclerosis (ALS) and Alzheimer's disease. In ALS, TDP-43 inclusions are typically also immunoreactive for fused in sarcoma (FUS)...
2017: PloS One
https://www.readbyqxmd.com/read/28304311/frontotemporal-dementia-due-to-the-novel-grn-arg161glyfsx36-mutation
#17
Stefano Gazzina, Silvana Archetti, Antonella Alberici, Elisa Bonomi, Maura Cosseddu, Diego Di Lorenzo, Alessandro Padovani, Barbara Borroni
Progranulin is a multifunctional growth factor mainly expressed in neurons and microglia. Loss-of-function mutations in the Granulin (GRN) gene are causative of frontotemporal dementia with TAR DNA-binding protein-43 inclusions. We reported the case of a 51-year-old male patient affected by sporadic agrammatic variant of primary progressive aphasia, in whom we identified a novel heterozygous deletion in the exon 6 (g.10338_39delAG, p.Arg161GlyfsX36). Plasma progranulin levels were significantly reduced and in silico analysis predicted a premature termination codon...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28300056/imbalance-between-tnf%C3%AE-and-progranulin-contributes-to-memory-impairment-and-anxiety-in-sleep-deprived-mice
#18
Kun Zhang, Yu-Jiao Li, Dan Feng, Peng Zhang, Ya-Tao Wang, Xiang Li, Shui-Bing Liu, Yu-Mei Wu, Ming-Gao Zhao
Sleep disorder is becoming a widespread problem in current society, and is associated with impaired cognition and emotional disorders. Progranulin (PGRN), also known as granulin epithelin precursor, promotes neurite outgrowth and cell survival, and is encoded by the GRN gene. It is a tumor necrosis factor α receptor (TNFR) ligand which is implicated in many central nervous system diseases. However, the role PGRN in sleep disorder remains unclear. In the present study, we found that sleep deprivation (S-DEP) impaired the memory and produced thigmotaxis/anxiety-like behaviors in mice...
March 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28293019/novel-adipokines-methodological-utility-in-human-obesity-research
#19
F Eichelmann, N Rudovich, A F Pfeiffer, M B Schulze, R di Giuseppe, H Boeing, K Aleksandrova
BACKGROUND: Adipokines could pose a link between adiposity, systemic inflammation and metabolic disease risk. However, it is unclear whether representative biomarkers are methodologically suitable for use in human obesity research. METHODS: We assessed the intra-individual reproducibility of selected adipokines in a sample of 207 apparently healthy participants with available biosample collections over 4 month period. Concentrations of the following adipokines were measured at each sampling time point: fatty-acid binding protein-4 (FABP-4), lipocalin-2, monocyte chemoattractant protein 1 (MCP-1), procalcitonin, progranulin, vaspin and visfatin/Nampt...
March 15, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28285794/missense-mutation-in-grn-gene-affecting-rna-splicing-and-plasma-progranulin-level-in-a-family-affected-by-frontotemporal-lobar-degeneration
#20
Simona Luzzi, Lara Colleoni, Paola Corbetta, Sara Baldinelli, Chiara Fiori, Francesca Girelli, Mauro Silvestrini, Paola Caroppo, Giorgio Giaccone, Fabrizio Tagliavini, Giacomina Rossi
Gene coding for progranulin, GRN, is a major gene linked to frontotemporal lobar degeneration. While most of pathogenic GRN mutations are null mutations leading to haploinsufficiency, GRN missense mutations do not have an obvious pathogenicity, and only a few have been revealed to act through different pathogenetic mechanisms, such as cytoplasmic missorting, protein degradation, and abnormal cleavage by elastase. The aim of this study was to disclose the pathogenetic mechanisms of the GRN A199V missense mutation, which was previously reported not to alter physiological progranulin features but was associated with a reduced plasma progranulin level...
June 2017: Neurobiology of Aging
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