Research Topics
| JAMES ROBERT CONNORSummaryAffiliation: Pennsylvania State University Country: USA Publications
Research Grants
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Publications
Profile of altered brain iron acquisition in restless legs syndromeJames R Connor
Department of Neurosurgery H110, G M Leader Family Laboratory for Alzheimer s Disease Research, Penn State College of Medicine, 500 University Dr, Hershey, PA 17033, USA
Brain 134:959-68. 2011..The data herein provide evidence for regulation of iron uptake and storage within brain microvessels that challenge the existing paradigm that the blood-brain barrier is merely a transport system...
Comparative study of the influence of Thy1 deficiency and dietary iron deficiency on dopaminergic profiles in the mouse striatumJames R Connor
Department of Neurosurgery, MS Hershey Medical Center, Penn State College of Medicine, Hershey, PA 17033, USA
J Neurosci Res 86:3194-202. 2008..Furthermore, the results reveal consistent differences in the dopaminergic profile in Thy1 knockout mice compared with iron-deficient mice, indicating that the effects of iron deficiency are not due only to a change in Thy1 expression...
Pathophysiology of restless legs syndrome: evidence for iron involvementJames R Connor
G M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University, Milton S Hershey Medical Center, 500 University Drive, PO Box 850, Hershey, PA 17033, USA
Curr Neurol Neurosci Rep 8:162-6. 2008..These data are reviewed and related to the dopaminergic system because of the use of dopamine agents in treating restless legs syndrome...- Altered dopaminergic profile in the putamen and substantia nigra in restless leg syndromeJames R Connor
Department of Neurosurgery, Penn State College of Medicine, Hershey, PA 17033 0850, USA
Brain 132:2403-12. 2009..The results are consistent with the hypothesis that a primary iron insufficiency produces a dopaminergic abnormality characterized as an overly activated dopaminergic system as part of the RLS pathology... - HFE mutations and Alzheimer's diseaseJames R Connor
Department of Neurosurgery, G M Leader Family Laboratory for Alzheimer s Disease Research, The Pennsylvania State University College of Medicine, Hershey, PA 17033, USA
J Alzheimers Dis 10:267-76. 2006..This review summarizes the data on HFE mutations in neurodegenerative disorders and what is known about HFE in the brain and the cell biology underlying the HFE mutation... - Consequences of expressing mutants of the hemochromatosis gene (HFE) into a human neuronal cell line lacking endogenous HFESang Y Lee
Department of Neurosurgery, Pennsylvania State University College of Medicine, Hershey, PA 17033 0850, USA
FASEB J 21:564-76. 2007..The results also raise multiple novel questions for future study about the function of the HFE protein... - Plasma biomarkers associated with ALS and their relationship to iron homeostasisRyan M Mitchell
George M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University College of Medicine, 500 University Drive H110, Hershey, PA 17033 0850, USA
Muscle Nerve 42:95-103. 2010..The data demonstrate a plasma biomarker profile in ALS patients that may differ from published reports of cerebrospinal fluid biomarkers... - Iron, the substantia nigra and related neurological disordersAmanda M Snyder
Department of Neurosurgery, Penn State University, M S Hershey Medical Center, Hershey, PA 17033, USA
Biochim Biophys Acta 1790:606-14. 2009..Nonetheless, that iron can influence neuronal cell death and dopamine function is clear... - A diet-induced mouse model for glutaric aciduria type IWilliam J Zinnanti
Department of Neurosurgery, Penn State College of Medicine, Hershey, PA 17033, USA
Brain 129:899-910. 2006..Thus, the Gcdh-/- mouse exposed to high protein or lysine may be a useful model of human GA-1 including developmentally dependent striatal vulnerability... - Tim-2 is the receptor for H-ferritin on oligodendrocytesBozho Todorich
Department of Neurosurgery, Penn State College of Medicine, Hershey, PA 17033 0850, USA
J Neurochem 107:1495-505. 2008..Taken together, these data provide compelling evidence that Tim-2 is the H-ferritin receptor on oligodendrocytes suggesting it is the primary mechanism for iron acquisition by these cells... - CSF proteomic analysis reveals persistent iron deficiency-induced alterations in non-human primate infantsRamaz N Geguchadze
Department of Neurosurgery, The Pennsylvania State University College of Medicine, Milton S Hershey Medical Center, Hershey, Pennsylvania 17033, USA
J Neurochem 105:127-36. 2008..Furthermore, alterations in the CSF proteome despite normal hematologic parameters indicate that there is a hierarchical system that prioritizes repletion of red cell mass at the expense of the brain... - Pre- and postweaning iron deficiency alters myelination in Sprague-Dawley ratsJohn L Beard
Nutrition Sciences, Integrative Biosciences Graduate Program, Pennsylvania State University, S 126 Henderson Building, University Park, PA 16802, USA
Dev Neurosci 25:308-15. 2003..This suggests that iron deficiency in later life, as well as during early postnatal growth, can effect the production and maintenance of myelin... - Brain iron uptake in hypotransferrinemic mice: influence of systemic iron statusJohn L Beard
Department of Nutritional Science, Nutrition Science Option, Pennsylvania State University, University Park, PA 16802, USA
J Neurosci Res 79:254-61. 2005..The proteins and mechanisms responsible for additional iron uptake into specific regions, or perhaps the redistribution are unclear though the data are supportive of a non-transferrin-bound iron uptake pathway... - Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type IWilliam J Zinnanti
Jake Gittlen Cancer Research Foundation, Department of Pathology, Penn State College of Medicine, Hershey, Pennsylvania 17033, USA
J Clin Invest 117:3258-70. 2007..Blocking brain lysine uptake reduced glutaric acid levels and brain injury. These findings provide what we believe are new monitoring and treatment strategies that may translate for use in human GA-I... - Metabolic analysis of mouse brains that have compromised iron storageAmanda M Ill
Department of Neurosurgery, Penn State University College of Medicine, M.S. Hershey Medical Center, Hershey, PA 17033, USA
Metab Brain Dis 21:77-87. 2006..Because H-ferritin is predominantly expressed in neurons, the neurochemical profile is suggestive of neuronal iron deficiency and may have relevance to the functional consequences associated with brain iron deficiency... - Cytokine toxicity to oligodendrocyte precursors is mediated by ironXuesheng Zhang
Department of Neurosurgery, Pennsylvania State University, College of Medicine Hershey, PA 17033, USA
Glia 52:199-208. 2005..A better understanding of these agents and the contribution of cellular iron status to cytokine toxicity may help develop a more consistent intervention strategy... - Perinatal nutritional iron deficiency permanently impairs hippocampus-dependent trace fear conditioning in ratsMatthew D McEchron
Department of Neural and Behavioral Science, Pennsylvania State University College of Medicine, Hershey, PA 17033, USA
Nutr Neurosci 8:195-206. 2005..The ID-induced learning impairments in this study, however, were not due to altered motor activity because learning was assessed using non-motor heart rate responses... - Moderate iron deficiency in infancy: biology and behavior in young ratsJohn L Beard
Department of Nutrition, Penn State University, PA 16802, USA
Behav Brain Res 170:224-32. 2006..Similar developmental delays in iron deficient human infants suggest to us that alterations in iron status during this developmental period likely affects developing brain monaminergic systems in these infants... - Myelin breakdown in Alzheimer's disease: a commentaryJames R Connor
Department of Neuroscience and Anatomy, M.S. Hershey Medical Center, Penn State University College of Medicine, Hershey, PA 17000, USA
Neurobiol Aging 25:45-7. 2004 - Influence of HFE variants and cellular iron on monocyte chemoattractant protein-1Ryan M Mitchell
George M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University College of Medicine Milton S Hershey Medical Center, Hershey, PA 17033, USA
J Neuroinflammation 6:6. 2009..We tested the hypothesis that the two most common HFE variants, H63D and C282Y, would affect cellular secretion of cytokines and trophic factors... - Iron transport proteins in the diseased brainJames R Connor
Department of Neuroscience and Anatomy, The Milton S. Hershey Medical Center, P.O. Box 850, PA 17033, USA
J Neurol Sci 207:112-3. 2003 - Iron status and neural functioningJohn L Beard
Department of Nutritional Sciences The Pennsylvania State University, University Park, Pennsylvania 16802, USA
Annu Rev Nutr 23:41-58. 2003..Future studies with more specific measures of neurodevelopment in iron-deficient human infants, and animal models, will allow investigators to more clearly define causal roles of brain iron in neural development and functioning... - Detection of cancer cells in the cerebrospinal fluid: current methods and future directionsCody L Weston
Department of Neurosurgery H110, Penn State University M, S, Hershey Medical Center, 500 University Dr, Hershey, PA, 17033 0850, USA
Fluids Barriers CNS 8:14. 2011..Furthermore, many cell-based detection methods can be combined with therapeutic agents to serve multiple medical functions through a common targeting system... - Regulation, mechanisms and proposed function of ferritin translocation to cell nucleiKhristy J Thompson
Department of Neuroscience and Anatomy, Pennsylvania State University College of Medicine, Milton S Hershey Medical Center, 17033, USA
J Cell Sci 115:2165-77. 2002..The knowledge generated as a result of these studies will also improve our understanding of iron-induced damage of nuclear constituents... - Prolyl-peptidyl isomerase, Pin1, phosphorylation is compromised in association with the expression of the HFE polymorphic allele, H63DEric C Hall
George M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University College of Medicine Milton S Hershey Medical Center, Hershey, PA 17033 0850, USA
Biochim Biophys Acta 1802:389-95. 2010..Thus, we have shown another cellular mechanism that HFE polymorphisms influence; further supporting their role as neurodegenerative disease modifiers... - Is ferroportin-hepcidin signaling altered in restless legs syndrome?Stacey L Clardy
Department of Neurosurgery, M S Hershey Medical Center, 500 University Drive, H110, Hershey, PA 17033, USA
J Neurol Sci 247:173-9. 2006..Nonetheless, these data support the mounting evidence that there is a biological basis for RLS and the underlying mechanism involves iron management... - Nuclear ferritin: A new role for ferritin in cell biologyAhmed A Alkhateeb
Department of Neurosurgery, Pennsylvania State University College of Medicine, Milton S Hershey Medical Center, Hershey, PA 17033, USA
Biochim Biophys Acta 1800:793-7. 2010.... - Oligodendrocytes and myelination: the role of ironBozho Todorich
Department of Neurosurgery, Pennsylvania State University College of Medicine, 500 University Drive, Hershey, PA 17033 0850, USA
Glia 57:467-78. 2009..It is the purpose of this review to give a comprehensive overview of the existing literature on role of iron in oligodendrocytes and the mechanisms of iron acquisition and intracellular handling... - Mitochondrial ferritin in the substantia nigra in restless legs syndromeAmanda M Snyder
Department of Neurosurgery, Pennsylvania State University College of Medicine, Hershey, Pennsylvania 17033, USA
J Neuropathol Exp Neurol 68:1193-9. 2009.... - Regulation of the profile of iron-management proteins in brain microvasculatureJoseph R Burdo
Department of Neural and Behavioral Sciences, Pennsylvania State College of Medicine, Hershey, Pennsylvania 17033, USA
J Cereb Blood Flow Metab 24:67-74. 2004..The results also indicate that brain iron status dictates the response of the microvascular protein profile to systemic iron manipulation... - Characterization of nuclear ferritin and mechanism of translocationNodar Surguladze
Department of Neurosurgery, The Pennsylvania State University College of Medicine, 500 University Drive, Hershey, PA 17033, USA
Biochem J 388:731-40. 2005..The picture that emerges is one in which ferritin translocation between the cytoplasm and the nucleus is post-translationally regulated and responds to environmental and nutritional cues... - Regulation of Hfe by stress factors in BV-2 cellsSang Y Lee
Department of Neurosurgery, H110, G M Leader Family Laboratory for Alzheimer s Disease Research, M S Hershey Medical Center, The Pennsylvania State University College of Medicine, 500 University Drive, Hershey, PA 17033 0850, USA
Neurobiol Aging 26:803-12. 2005..These results are the first in a series of studies to understand how mutations in Hfe can be a risk factor for AD... - Interactions and reactions of ferritin with DNANodar Surguladze
Departments of Neural and Behavioral Science and Biochemistry and Molecular Biology, The Pennsylvania State University College of Medicine, Hershey, Pennsylvania 17033, USA
J Biol Chem 279:14694-702. 2004..These results suggest that the release of iron from ferritin is an important part of the nicking mechanism. The potential role of ferritin as a protector of the genome is discussed in the context of these results... - Ferritin subunits in CSF are decreased in restless legs syndromeStacey L Clardy
Department of Neurosurgery, M.S. Hershey Medical Center, Hershey, Pennsylvania 17033, USA
J Lab Clin Med 147:67-73. 2006..These data suggest that CSF ferritin levels may provide a biomarker for assisting in the diagnosis of RLS... - Efficacy of interleukin-13 receptor-targeted liposomal doxorubicin in the intracranial brain tumor modelAchuthamangalam B Madhankumar
Department of Neurosurgery H110, G M Leader Family Laboratory, Penn State University, College of Medicine, M S Hershey Medical Center, 500 University Drive, Hershey, PA 17033 0850, USA
Mol Cancer Ther 8:648-54. 2009..These data show that IL-13 targeted nanovesicles are a viable option for the treatment of brain tumors... - MRI and histological analysis of beta-amyloid plaques in both human Alzheimer's disease and APP/PS1 transgenic miceMark D Meadowcroft
Department of Radiology Center for NMR Research, Pennsylvania State University College of Medicine, Milton S Hershey Medical Center, Hershey, Pennsylvania, USA
J Magn Reson Imaging 29:997-1007. 2009.... - Brain iron uptake and homeostatic mechanisms: an overviewJoseph R Burdo
Department of Neuroscience and Anatomy, Penn State University College of Medicine, M.S. Hershey Medical Center, Hershey, PA 17033, USA
Biometals 16:63-75. 2003.... - Altered eyeblink reflex conditioning in restless legs syndrome patientsMatthew D McEchron
Department of Neural and Behavioral Sciences, Pennsylvania State University College of Medicine, Hershey, PA 17033, USA
Sleep Med 11:314-9. 2010..This led us to determine if RLS patients show alterations in a sensory-motor reflex conditioning task called differential eyeblink conditioning... - Differential expression of genes in amyotrophic lateral sclerosis revealed by profiling the post mortem cortexXin-Sheng Wang
Department of Neurosurgery, Penn State College of Medicine, Hershey Medical Center, Hershey, PA 17033, USA
Amyotroph Lateral Scler 7:201-10. 2006.... - Subcellular localization of iron regulatory proteins to Golgi and ER membranesStephanie M Patton
Department of Neurosurgery, G M Leader Family Laboratory for Alzheimer s Disease Research, Pennsylvania State University College of Medicine, Hershey, PA 17033, USA
J Cell Sci 118:4365-73. 2005..As a result of this study we propose a novel model for intracellular distribution of IRPs and identify differences between the two iron regulatory proteins... - Interleukin-13 receptor-targeted nanovesicles are a potential therapy for glioblastoma multiformeA B Madhankumar
Department of Neurosurgery (H110, G.M. Leader Family Laboratory for Alzheimer's Disease Research, Milton S. Hershey Medical Center, Penn State University, 500 University Drive, Hershey, PA 17033-0850, USA
Mol Cancer Ther 5:3162-9. 2006..These results strongly suggest that IL-13-conjugated liposomes carrying cytotoxic agents are a feasible approach for creating a nanovesicle drug delivery system for brain tumor therapy... - Development of a fluorescent reporter to assess iron regulatory protein activity in living cellsRebecca J Henderson
Department of Pharmacology, College of Medicine, Pennsylvania State University, 500 University Drive, Hershey, Pennsylvania 17033, USA
Biochim Biophys Acta 1743:162-8. 2005.... - A light and electron microscopic study of divalent metal transporter-1 distribution in the rat hippocampus, after kainate-induced neuronal injuryXin Sheng Wang
Department of Anatomy, National University of Singapore, 119260, Singapore
Exp Neurol 177:193-201. 2002..The observation that DMT-1 is present on astrocytic end feet in contact with blood vessels suggests that these cells may be involved in uptake of iron from endothelial cells... - Abnormal iron delivery to the bone marrow in neonatal hypotransferrinemic miceAtsushi Takeda
Department of Radiobiochemistry, School of Pharmaceutical Sciences, University of Shizuoka, Yada, Japan
Biometals 15:33-6. 2002..However, the iron seems to be unavailable for the production of hemoglobin. These results suggest that transferrin-dependent iron uptake by erythroid cells in the bone marrow is essential for the development of erythrocytes... - Myelination and motor coordination are increased in transferrin transgenic miceMaria-Carla Saleh
, Institut Pasteur, Paris, France
J Neurosci Res 72:587-94. 2003..Our findings indicate that, besides its known iron transport function, Tf is able to influence myelination process and induce behavioral improvements in mice... - Upregulation of iron regulatory proteins and divalent metal transporter-1 isoforms in the rat hippocampus after kainate induced neuronal injuryEn Huang
Department of Anatomy, National University of Singapore, Singapore 119260, Singapore
Exp Brain Res 170:376-86. 2006..This could lead to increased iron influx into the brain areas undergoing neurodegeneration, and might be a factor contributing to neuronal damage after the initial excitotoxic injury... - The lipophilic iron compound TMH-ferrocene [(3,5,5-trimethylhexanoyl)ferrocene] increases iron concentrations, neuronal L-ferritin, and heme oxygenase in brains of BALB/c miceElise A Malecki
Department of Neuroscience and Anatomy, College of Medicine, The Pennsylvania State University, Hershey 17033, USA
Biol Trace Elem Res 86:73-84. 2002.... - Iron deficiency alters iron regulatory protein and iron transport protein expression in the perinatal rat brainAsha Jyothi M Siddappa
Division of Neonatology, Department of Pediatrics, University of Minnesota Medical School, Minneapolis 55455, USA
Pediatr Res 53:800-7. 2003..Brain iron distribution during ID seems to be locally rather than globally regulated... - Developmental changes in the expression of iron regulatory proteins and iron transport proteins in the perinatal rat brainAsha Jyothi M Siddappa
Division of Neonatology, Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA
J Neurosci Res 68:761-75. 2002..These developmental changes in IRP and transporter expression suggest potentially different time periods of brain structure vulnerability to iron deficiency or iron overload... - Gene expression of transferrin and transferrin receptor in brains of control vs. iron-deficient ratsJian Han
Department of Nutrition, The Pennsylvania State University, S 126, Henderson Building, University Park, PA 16802, USA
Nutr Neurosci 6:1-10. 2003..This capacity may be associated with increased iron-Tf uptake from plasma, stabilization of TfR mRNA, or increased Tf mRNA translation efficiency in specific cell types within the brain... - Persistent neurochemical and behavioral abnormalities in adulthood despite early iron supplementation for perinatal iron deficiency anemia in ratsBarbara T Felt
Center for Human Growth and Development and Department of Pediatrics and Communicable Diseases, University of Michigan, Ann Arbor, 48109, USA
Behav Brain Res 171:261-70. 2006..We recently reported that rats with chronic perinatal IDA, had altered regional brain iron, monoamines, and sensorimotor skill emergence during early development... - New insights for glutaric aciduria type IWilliam J Zinnanti
Brain 129:e55. 2006 - H and L ferritin subunit mRNA expression differs in brains of control and iron-deficient ratsJian Han
Department of Nutrition, The Pennsylvania State University, University Park, PA 16802, USA
J Nutr 132:2769-74. 2002..All of these data suggest that regulation of ferritin subunits is cellular and/or regional specific... - Hypoxia-inducible factor prolyl 4-hydroxylase inhibition. A target for neuroprotection in the central nervous systemAmbreena Siddiq
Department of Neurology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, Massachusetts 02115, USA
J Biol Chem 280:41732-43. 2005..Taken together these findings identified low molecular weight and peptide HIF prolyl 4-hydroxylase inhibitors as novel neurological therapeutics for stroke as well as other diseases associated with oxidative stress... - Microscopic R2* mapping of reduced brain iron in the Belgrade ratHolly A Zywicke
Laboratory of Diagnostic Radiology Research, Clinical Center, National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, MD, USA
Ann Neurol 52:102-5. 2002..Therefore, for the clinical monitoring of movement disorders for which normal brain iron homeostasis (either overload or reduction) may be implicated, R2* mapping appears to be well-suited... - Ferritin levels in the cerebrospinal fluid and restless legs syndrome: effects of different clinical phenotypesChristopher J Earley
Department of Neurology, Johns Hopkins School of Medicine, Johns Hopkins Bayview Medical Center, Baltimore, MD 21224, USA
Sleep 28:1069-75. 2005.... - Alteration of iron regulatory proteins (IRP1 and IRP2) and ferritin in the brains of scrapie-infected miceBoe Hyun Kim
Ilsong Institute of Life Science, Academy of Science, Hallym University, Dongan Gu, Anyang, Kyounggi do 431 060, South Korea
Neurosci Lett 422:158-63. 2007..These results suggest that alteration of iron metabolism contributes to development of neurodegeneration and that some protective mechanisms against iron-induced oxidative damage may occur during the pathogenesis of TSEs... - Nitric oxide protects astrocytes from oxidative stressSara J Robb
Department of Physiology, University College London, Gower Street, London, United Kingdom
Ann N Y Acad Sci 962:93-102. 2002..Taken together, these data suggest that nitric oxide can act as a powerful antioxidant for astrocytes preserving mitochondrial and cellular integrity during oxidative stress... - Redox metals in Alzheimer's diseaseBozho M Todorich
George M. Leader Family Laboratory for Alzheimer's Disease Research, Department of Neural and Behavioral Sciences, Pennsylvania State University School of Medicine, Hershey 17033, USA
Ann N Y Acad Sci 1012:171-8. 2004..Iron, copper, and zinc are metals of special interest in the pathogenesis of these disorders. This review will focus primarily on iron... - Iron, brain ageing and neurodegenerative disordersLuigi Zecca
Institute of Biomedical Technologies Italian National Research Council, 20090 Segrate, Milano, Italy
Nat Rev Neurosci 5:863-73. 2004..By studying the accumulation and cellular distribution of iron during ageing, we should be able to increase our understanding of these neurodegenerative disorders and develop new therapeutic strategies...
Research Grants
- Isolation and Characterization of a Ferritin ReceptorJames Connor; Fiscal Year: 2005..This project will significantly alter our thinking on brain iron transport and open novel directions for research in oligodendrocyte biology and diagnositic and therapeutic strategies in demyelinating disorders ..
- Iron Acquisition Mechanisms in OligodendrocytesJames Connor; Fiscal Year: 2009..abstract_text> ..
- Iron Acquisition Mechanisms in OligodendrocytesJAMES ROBERT CONNOR; Fiscal Year: 2010..abstract_text> ..
- Translocation and Function of Nuclear FerritinJames Connor; Fiscal Year: 2004..abstract_text> ..