James R Connor

Summary

Affiliation: Pennsylvania State University
Country: USA

Publications

  1. ncbi request reprint Decreased transferrin receptor expression by neuromelanin cells in restless legs syndrome
    J R Connor
    Department of Neural and Behavior Sciences, G M Leader Family Laboratory for AD Research, Penn Penn State College of Medicine, Hershey, PA 17033, USA
    Neurology 62:1563-7. 2004
  2. ncbi request reprint Iron transport proteins in the diseased brain
    James 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
  3. ncbi request reprint Neuropathological examination suggests impaired brain iron acquisition in restless legs syndrome
    J R Connor
    Department of Neuroscience and Anatomy, Penn State University College of Medicine, M S Hershey Medical Center, Hershey, PA 17033, USA
    Neurology 61:304-9. 2003
  4. ncbi request reprint Myelin breakdown in Alzheimer's disease: a commentary
    James 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
  5. doi request reprint Expression of the HFE allelic variant H63D in SH-SY5Y cells affects tau phosphorylation at serine residues
    Eric C Hall
    George M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University College of Medicine Milton S Hershey Medical Center, Hershey, PA 17033, USA
    Neurobiol Aging 32:1409-19. 2011
  6. doi request reprint Efficacy of interleukin-13 receptor-targeted liposomal doxorubicin in the intracranial brain tumor model
    Achuthamangalam 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
  7. ncbi request reprint Consequences of expressing mutants of the hemochromatosis gene (HFE) into a human neuronal cell line lacking endogenous HFE
    Sang Y Lee
    Department of Neurosurgery, Pennsylvania State University College of Medicine, Hershey, PA 17033 0850, USA
    FASEB J 21:564-76. 2007
  8. doi request reprint Tim-2 is the receptor for H-ferritin on oligodendrocytes
    Bozho Todorich
    Department of Neurosurgery, Penn State College of Medicine, Hershey, PA 17033 0850, USA
    J Neurochem 107:1495-505. 2008
  9. doi request reprint Plasma biomarkers associated with ALS and their relationship to iron homeostasis
    Ryan 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
  10. doi request reprint HFE polymorphisms affect cellular glutamate regulation
    Ryan M Mitchell
    George M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University College of Medicine Milton S Hershey Medical Center, 500 University Drive H110, Hershey, PA 17033, USA
    Neurobiol Aging 32:1114-23. 2011

Collaborators

Detail Information

Publications97

  1. ncbi request reprint Decreased transferrin receptor expression by neuromelanin cells in restless legs syndrome
    J R Connor
    Department of Neural and Behavior Sciences, G M Leader Family Laboratory for AD Research, Penn Penn State College of Medicine, Hershey, PA 17033, USA
    Neurology 62:1563-7. 2004
    ..Its etiology is unknown, but MRI analyses and immunohistochemical studies on autopsy tissue suggest the substantia nigra (SN) of patients with RLS has subnormal amounts of iron...
  2. ncbi request reprint Iron transport proteins in the diseased brain
    James 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
  3. ncbi request reprint Neuropathological examination suggests impaired brain iron acquisition in restless legs syndrome
    J R Connor
    Department of Neuroscience and Anatomy, Penn State University College of Medicine, M S Hershey Medical Center, Hershey, PA 17033, USA
    Neurology 61:304-9. 2003
    ..To assess neuropathology in individuals with restless legs syndrome (RLS)...
  4. ncbi request reprint Myelin breakdown in Alzheimer's disease: a commentary
    James 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
  5. doi request reprint Expression of the HFE allelic variant H63D in SH-SY5Y cells affects tau phosphorylation at serine residues
    Eric C Hall
    George M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University College of Medicine Milton S Hershey Medical Center, Hershey, PA 17033, USA
    Neurobiol Aging 32:1409-19. 2011
    ..These results suggest H63D HFE increases tau phosphorylation via GSK-3β activity and iron-mediated oxidative stress...
  6. doi request reprint Efficacy of interleukin-13 receptor-targeted liposomal doxorubicin in the intracranial brain tumor model
    Achuthamangalam 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...
  7. ncbi request reprint Consequences of expressing mutants of the hemochromatosis gene (HFE) into a human neuronal cell line lacking endogenous HFE
    Sang 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...
  8. doi request reprint Tim-2 is the receptor for H-ferritin on oligodendrocytes
    Bozho 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...
  9. doi request reprint Plasma biomarkers associated with ALS and their relationship to iron homeostasis
    Ryan 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...
  10. doi request reprint HFE polymorphisms affect cellular glutamate regulation
    Ryan M Mitchell
    George M Leader Family Laboratory, Department of Neurosurgery, Pennsylvania State University College of Medicine Milton S Hershey Medical Center, 500 University Drive H110, Hershey, PA 17033, USA
    Neurobiol Aging 32:1114-23. 2011
    ..This study demonstrates HFE cellular effects that extend beyond iron regulation, and suggests that H63D HFE may promote glutamate toxicity...
  11. pmc Influence of HFE variants and cellular iron on monocyte chemoattractant protein-1
    Ryan 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...
  12. pmc Mutant HFE H63D protein is associated with prolonged endoplasmic reticulum stress and increased neuronal vulnerability
    Yiting Liu
    Department of Neurosurgery, Pennsylvania State University, Milton S Hershey Medical Center, Hershey, Pennsylvania 17033, USA
    J Biol Chem 286:13161-70. 2011
    ..Overall, our studies indicate that the HFE H63D mutant protein is associated with prolonged ER stress and chronically increased neuronal vulnerability...
  13. pmc Mitochondrial ferritin in the substantia nigra in restless legs syndrome
    Amanda M Snyder
    Department of Neurosurgery, Pennsylvania State University College of Medicine, Hershey, Pennsylvania 17033, USA
    J Neuropathol Exp Neurol 68:1193-9. 2009
    ....
  14. pmc Mechanism of age-dependent susceptibility and novel treatment strategy in glutaric acidemia type I
    William 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...
  15. ncbi request reprint HFE polymorphisms influence the response to chemotherapeutic agents via induction of p16INK4A
    Sang Y Lee
    Department of Neurosurgery, The Pennsylvania State University College of Medicine, MS Hershey Medical Center, Hershey, PA 17033 0850, USA
    Int J Cancer 129:2104-14. 2011
    ....
  16. doi request reprint Prolyl-peptidyl isomerase, Pin1, phosphorylation is compromised in association with the expression of the HFE polymorphic allele, H63D
    Eric 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...
  17. doi request reprint H63D HFE polymorphisms are associated with increased disease duration and decreased muscle superoxide dismutase-1 expression in amyotrophic lateral sclerosis patients
    Xiaowei W Su
    George M Leader Family Laboratory, Department of Neurosurgery, The Pennsylvania State University College of Medicine, 500 University Drive H110, Hershey, Pennsylvania 17033 0850, USA
    Muscle Nerve 48:242-6. 2013
    ..Investigating the physiological alterations induced by H63D polymorphisms in ALS patients may elucidate mechanisms by which this genotype alters disease...
  18. pmc Altered dopaminergic profile in the putamen and substantia nigra in restless leg syndrome
    James 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...
  19. doi request reprint A mutation in the HFE gene is associated with altered brain iron profiles and increased oxidative stress in mice
    Wint Nandar
    Department of Neurosurgery, The Pennsylvania State University, M S Hershey Medical Center, PA 17033, USA
    Biochim Biophys Acta 1832:729-41. 2013
    ..The H67D knock-in mouse can be used as a model to evaluate how the H63D HFE mutation contributes to neurodegenerative diseases...
  20. doi request reprint Comparative study of the influence of Thy1 deficiency and dietary iron deficiency on dopaminergic profiles in the mouse striatum
    James 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...
  21. pmc Profile of altered brain iron acquisition in restless legs syndrome
    James 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...
  22. pmc Postmortem and imaging based analyses reveal CNS decreased myelination in restless legs syndrome
    James R Connor
    Department of Neurosurgery, Pennsylvania State University College of Medicine, Milton S Hershey Medical Center, Hershey, PA 17033 0850, USA
    Sleep Med 12:614-9. 2011
    ..Iron deficiency is associated with hypomyelination in brains of animals. Therefore we hypothesized that a myelin deficit should be present in the brains of patients with RLS...
  23. ncbi request reprint 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...
  24. doi request reprint Quantitative proteomic analyses of cerebrospinal fluid using iTRAQ in a primate model of iron deficiency anemia
    Stephanie M Patton
    Penn State University College of Medicine, Hershey, PA, USA
    Dev Neurosci 34:354-65. 2012
    ..The compromised brain infrastructure could account for observations of behavioral deficits in children during and after the period of anemia...
  25. doi request reprint H-ferritin is the major source of iron for oligodendrocytes
    Bozho Todorich
    Department of Neurosurgery, Pennsylvania State University College of Medicine, Hershey, Pennsylvania, USA
    Glia 59:927-35. 2011
    ..Given that ferritin receptors, but not transferrin receptors can be demonstrated on oligodendrocytes in vivo, the delivery of iron to oligodendrocytes via ferritin may be the more biological relevant delivery system...
  26. doi request reprint The mechanism of vanadium-mediated developmental hypomyelination is related to destruction of oligodendrocyte progenitors through a relationship with ferritin and iron
    Bozho Todorich
    Department of Neurosurgery, Pennsylvania State University College of Medicine, M S Hershey Medical Center, 500 University Drive, Hershey, PA 17033 0850, USA
    Neurotox Res 19:361-73. 2011
    ..Therefore, depletion of OPCs due to vanadium exposure in early post-natal period may be an important mechanism of vanadium-induced hypomyelination...
  27. doi request reprint HFE gene variants affect iron in the brain
    Wint Nandar
    Department of Neurosurgery, Pennsylvania State University, M S Hershey Medical Center, Hershey, PA 17033, USA
    J Nutr 141:729S-739S. 2011
    ..Importantly, the data herein also begin to dispel the long-held view that the brain is protected from iron accumulation associated with the HFE mutations...
  28. doi request reprint Heavy chain ferritin siRNA delivered by cationic liposomes increases sensitivity of cancer cells to chemotherapeutic agents
    Xiaoli Liu
    Department of Neurosurgery, Penn State Cancer Institute, Pennsylvania State University College of Medicine, Milton S Hershey Medical Center, Hershey, Pennsylvania 17033, USA
    Cancer Res 71:2240-9. 2011
    ..Overall, our results illustrate how silencing H-ferritin can effectively sensitize tumors to chemotherapy and also show the ability of C-liposomes to serve as a novel in vivo delivery tool for siRNAs...
  29. doi request reprint Iron, the substantia nigra and related neurological disorders
    Amanda 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...
  30. doi request reprint Regional and cellular distribution of mitochondrial ferritin in the mouse brain
    Amanda M Snyder
    Department of Neurosurgery, Pennsylvania State University College of Medicine, Hershey, PA 17033, USA
    J Neurosci Res 88:3133-43. 2010
    ..Given the importance and relationship between iron and mitochondrial activity, understanding the role of mitochondrial ferritin can be expected to contribute to our knowledge of mitochondrial dysfunction and neurodegenerative disease...
  31. ncbi request reprint Regulation of the profile of iron-management proteins in brain microvasculature
    Joseph 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...
  32. doi request reprint Functional roles of transferrin in the brain
    Dominique F Leitner
    Department of Neurosurgery, Penn State University, M S Hershey Medical Center, 500 University Dr, Hershey, PA 17033 0850, USA
    Biochim Biophys Acta 1820:393-402. 2012
    ..Finally, transferrin may play an important role in neurodegenerative diseases through its ability to mobilize iron...
  33. doi request reprint Oligodendrocytes and myelination: the role of iron
    Bozho 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...
  34. ncbi request reprint HFE mutations and Alzheimer's disease
    James 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...
  35. ncbi request reprint Subcellular localization of iron regulatory proteins to Golgi and ER membranes
    Stephanie 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...
  36. pmc MRI and histological analysis of beta-amyloid plaques in both human Alzheimer's disease and APP/PS1 transgenic mice
    Mark 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
    ....
  37. doi request reprint Nuclear ferritin: A new role for ferritin in cell biology
    Ahmed 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
    ....
  38. pmc Characterization of nuclear ferritin and mechanism of translocation
    Nodar 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...
  39. ncbi request reprint Cytokine toxicity to oligodendrocyte precursors is mediated by iron
    Xuesheng 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...
  40. doi request reprint The significance of ferritin in cancer: anti-oxidation, inflammation and tumorigenesis
    Ahmed A Alkhateeb
    Department of Neurosurgery, The Pennsylvania State University Hershey Medical Center, Hershey, PA, USA
    Biochim Biophys Acta 1836:245-54. 2013
    ..Moreover, we examine the literature on ferritin's contributions to tumor progression and therapy resistance, in addition to its therapeutic potential. ..
  41. pmc Proteomic analysis of the cerebrospinal fluid of patients with restless legs syndrome/Willis-Ekbom disease
    Stephanie M Patton
    Department of Neurosurgery, Penn State University, Milton S, Hershey Medical Center, 500 University Drive, Hershey, Pennsylvania PA 17033, USA
    Fluids Barriers CNS 10:20. 2013
    ..The purpose of this study was to determine if biomarkers in the cerebrospinal fluid can distinguish RLS/WED patients from neurological controls...
  42. ncbi request reprint Differential expression of genes in amyotrophic lateral sclerosis revealed by profiling the post mortem cortex
    Xin Sheng Wang
    Department of Neurosurgery, Penn State College of Medicine, Hershey Medical Center, Hershey, PA 17033, USA
    Amyotroph Lateral Scler 7:201-10. 2006
    ....
  43. ncbi request reprint CSF proteomic analysis reveals persistent iron deficiency-induced alterations in non-human primate infants
    Ramaz 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...
  44. ncbi request reprint Ferritin subunits in CSF are decreased in restless legs syndrome
    Stacey 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...
  45. ncbi request reprint Pre- and postweaning iron deficiency alters myelination in Sprague-Dawley rats
    John 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...
  46. ncbi request reprint Brain iron uptake in hypotransferrinemic mice: influence of systemic iron status
    John 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...
  47. ncbi request reprint Moderate iron deficiency in infancy: biology and behavior in young rats
    John 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...
  48. ncbi request reprint Iron status and neural functioning
    John 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...
  49. pmc Detection of cancer cells in the cerebrospinal fluid: current methods and future directions
    Cody 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...
  50. ncbi request reprint Iron deficiency alters H- and L-ferritin expression in rat brain
    J Han
    Department of Nutrition, The Pennsylvania State University, University Park 16802, USA
    Cell Mol Biol (Noisy-le-grand) 46:517-28. 2000
    ..These data indicate that ferritin H and L subunits within the brain respond differently to iron status and suggest post transcription regulation as a key event...
  51. ncbi request reprint Genetic heterogeneity of amyotrophic lateral sclerosis: implications for clinical practice and research
    Xiaowei W Su
    Department of Neurosurgery, The Pennsylvania State University College of Medicine, Hershey, Pennsylvania, USA
    Muscle Nerve 49:786-803. 2014
    ..Muscle Nerve 49: 786-803, 2014. ..
  52. doi request reprint Iron and ER stress in neurodegenerative disease
    Yiting Liu
    Department of Neurosurgery, M S Hershey Medical Center, Pennsylvania State University, Hershey, PA 17033, USA
    Biometals 25:837-45. 2012
    ..And, we discuss the roles of iron and HFE genotype in selective neuronal vulnerability and degeneration through modifying the ER stress level...
  53. ncbi request reprint Pathophysiology of restless legs syndrome: evidence for iron involvement
    James 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...
  54. ncbi request reprint Interactions and reactions of ferritin with DNA
    Nodar 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...
  55. ncbi request reprint Brain iron uptake and homeostatic mechanisms: an overview
    Joseph 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
    ....
  56. ncbi request reprint Distribution of divalent metal transporter 1 and metal transport protein 1 in the normal and Belgrade rat
    J R Burdo
    Department of Neuroscience and Anatomy, Hershey Medical Center, Pennsylvania State College of Medicine, 500 University Drive, Hershey, PA 17036, USA
    J Neurosci Res 66:1198-207. 2001
    ..These results indicate that DMT1 and MTP1 are involved in brain iron transport and this involvement is regionally and cellularly specific...
  57. doi request reprint Ferritin stimulates breast cancer cells through an iron-independent mechanism and is localized within tumor-associated macrophages
    Ahmed A Alkhateeb
    Department of Neurosurgery, The Pennsylvania State University Hershey Medical Center, Hershey, PA, USA
    Breast Cancer Res Treat 137:733-44. 2013
    ..Together, these findings suggest that the release of ferritin by infiltrating macrophages in breast tumors may represent an inflammatory effector mechanism by which ferritin directly stimulates tumorigenesis...
  58. ncbi request reprint Regulation of Hfe by stress factors in BV-2 cells
    Sang 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...
  59. doi request reprint Altered eyeblink reflex conditioning in restless legs syndrome patients
    Matthew 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...
  60. ncbi request reprint 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 mice
    Elise 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
    ....
  61. ncbi request reprint Development of a fluorescent reporter to assess iron regulatory protein activity in living cells
    Rebecca 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
    ....
  62. ncbi request reprint Metabolic analysis of mouse brains that have compromised iron storage
    Amanda 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...
  63. pmc Direct magnetic resonance imaging of histological tissue samples at 3.0T
    Mark D Meadowcroft
    Center for NMR Research, Department of Radiology, Pennsylvania State University College of Medicine, Milton S Hershey Medical Center, Hershey, Pennsylvania 17033, USA
    Magn Reson Med 57:835-41. 2007
    ..This work demonstrates the feasibility of using a microimaging histological coil to image thin slices of pathologically diseased tissue to obtain a precise one-to-one comparison between stained tissue and MR images...
  64. doi request reprint H63D mutation in hemochromatosis alters cholesterol metabolism and induces memory impairment
    Fatima Ali-Rahmani
    Department of Biochemistry and Molecular Biology, The Pennsylvania State University College of Medicine, Hershey, PA, USA Department of Neurosurgery, The Pennsylvania State University College of Medicine, Hershey, PA, USA
    Neurobiol Aging 35:1511.e1-12. 2014
    ..These results indicate that the alterations in cholesterol metabolism associated with expression of H63D-HFE may contribute to the development of AD. ..
  65. ncbi request reprint A diet-induced mouse model for glutaric aciduria type I
    William 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...
  66. ncbi request reprint Regulation, mechanisms and proposed function of ferritin translocation to cell nuclei
    Khristy 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...
  67. ncbi request reprint Perinatal nutritional iron deficiency permanently impairs hippocampus-dependent trace fear conditioning in rats
    Matthew 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...
  68. ncbi request reprint Interleukin-13 receptor-targeted nanovesicles are a potential therapy for glioblastoma multiforme
    A 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...
  69. ncbi request reprint Mechanisms and regulation of transferrin and iron transport in a model blood-brain barrier system
    J R Burdo
    Department of Neuroscience, Pennsylvania State College of Medicine, Mail Code H109, 500 University Drive, Hershey, PA 17033, USA
    Neuroscience 121:883-90. 2003
    ..These data have considerable implications for common neurodegenerative diseases that are associated with excess brain iron accumulation and the numerous neurological complications associated with brain iron deficiency...
  70. ncbi request reprint Demonstration and characterization of the iron regulatory protein in human brain
    J Hu
    Department of Neuroscience and Anatomy, Penn State University College of Medicine, M S Hershey Medical Center, Hershey, Pennsylvania 17033, USA
    J Neurochem 67:838-44. 1996
    ..This latter observation suggests the brain IRP undergoes posttranslational modification, the result of which may influence the stability of the IRE/IRP complex...
  71. ncbi request reprint Transferrin is required for normal distribution of 59Fe and 54Mn in mouse brain
    E A Malecki
    Department of Pediatrics, College of Medicine, The Pennsylvania State University, Hershey, PA 17033, USA
    J Neurol Sci 170:112-8. 1999
    ..Additionally, these data suggest that endogenous synthesis of Tf is necessary for Fe transport from the choroid plexus...
  72. ncbi request reprint The intracellular location of iron regulatory proteins is altered as a function of iron status in cell cultures and rat brain
    D J Pinero
    Department of Neuroscience and Anatomy, G M Leader Family Laboratory for Alzheimer s Disease Research, Penn State University College of Medicine, Hershey, PA 17033, USA
    J Nutr 131:2831-6. 2001
    ..The elucidation of this mechanism will have a significant impact on our understanding of eukaryotic gene regulation...
  73. ncbi request reprint Ferritin: a novel mechanism for delivery of iron to the brain and other organs
    J Fisher
    Dept of Neurosurgery, Pennsylvania State University, Milton S Hershey Medical Center, Hershey, PA 17033 0850, USA
    Am J Physiol Cell Physiol 293:C641-9. 2007
    ..The relative amount of iron that could be delivered via H-ferritin could make this protein a predominant player in cellular iron delivery...
  74. ncbi request reprint Cellular distribution of iron, transferrin, and ferritin in the hypotransferrinemic (Hp) mouse brain
    T K Dickinson
    George M Leader Family Laboratory for Alzheimer s Disease Research, Department of Neuroscience and Anatomy, Pennsylvania State University, College of Medicine, M S Hershey Medical Center, Hershey 17033, USA
    J Comp Neurol 355:67-80. 1995
    ....
  75. ncbi request reprint Histological analysis of selected brain regions of hypotransferrinemic mice
    T K Dickinson
    George M Leader Family Laboratory for Alzheimer s Disease Research, Department of Neuroscience and Anatomy, Pennsylvania State University, College of Medicine, M S Hershey Medical Center, Hershey 17033
    Brain Res 635:169-78. 1994
    ..The results of this initial study indicate the Hp animal may be an interesting model for investigating specific aspects of neural development and has considerable potential for examining the importance of iron regulation in the brain...
  76. ncbi request reprint Cellular iron status influences the functional relationship between microglia and oligodendrocytes
    X Zhang
    Department of Neurosurgery, College of Medicine, Pennsylvania State University, M S Hershey Medical Center, Hershey, PA 17033 0850, USA
    Glia 54:795-804. 2006
    ..The results further indicate that ferritin, released by microglia, is a significant source of iron for oligodendrocytes...
  77. ncbi request reprint cDNA cloning by amplification of circularized first strand cDNAs reveals non-IRE-regulated iron-responsive mRNAs
    Z Ye
    George M Leader Family Laboratory for Alzheimer s Disease Research, Pennsylvania State University College of Medicine, Hershey, Pennsylvania, 17033, USA
    Biochem Biophys Res Commun 275:223-7. 2000
    ..The data enabled us to exclude the possibility that the expression of these iron responsive genes are regulated by IREs...
  78. ncbi request reprint Characterization of a novel brain-derived microglial cell line isolated from neonatal rat brain
    P Cheepsunthorn
    Department of Neuroscience and Anatomy, M.S. Hershey Medical Center, Pennsylvania State University, College of Medicine, Hershey, Pennsylvania, USA
    Glia 35:53-62. 2001
    ..In addition, we demonstrate that the activation of microglial cells could be modified by iron...
  79. ncbi request reprint Iron and neurodegenerative disorders
    K J Thompson
    Department of Neuroscience and Anatomy, Pennsylvania State University College of Medicine, Milton S. Hershey Medical Center, Hershey, PA, USA
    Brain Res Bull 55:155-64. 2001
    ..The underlying pathogenic event in oxidative stress is cellular iron mismanagement...
  80. ncbi request reprint Iron and iron management proteins in neurobiology
    J R Connor
    Department of Neuroscience and Anatomy, Penn State University College of Medicine, M.S. Hershey Medical Center, Hershey, Pennsylvania 17033, USA
    Pediatr Neurol 25:118-29. 2001
    ....
  81. pmc Alteration of iron regulatory proteins (IRP1 and IRP2) and ferritin in the brains of scrapie-infected mice
    Boe 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...
  82. ncbi request reprint Myelination and motor coordination are increased in transferrin transgenic mice
    Maria Carla Saleh
    Unité d Expression des Gènes Eucaryotes, 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...
  83. ncbi request reprint Developmental changes in the expression of iron regulatory proteins and iron transport proteins in the perinatal rat brain
    Asha 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...
  84. ncbi request reprint A light and electron microscopic study of divalent metal transporter-1 distribution in the rat hippocampus, after kainate-induced neuronal injury
    Xin 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...
  85. ncbi request reprint New insights for glutaric aciduria type I
    William J Zinnanti
    Brain 129:e55. 2006
  86. pmc Persistent neurochemical and behavioral abnormalities in adulthood despite early iron supplementation for perinatal iron deficiency anemia in rats
    Barbara 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...
  87. ncbi request reprint Abnormal iron delivery to the bone marrow in neonatal hypotransferrinemic mice
    Atsushi 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...
  88. ncbi request reprint Iron deficiency alters iron regulatory protein and iron transport protein expression in the perinatal rat brain
    Asha 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...
  89. ncbi request reprint Upregulation of iron regulatory proteins and divalent metal transporter-1 isoforms in the rat hippocampus after kainate induced neuronal injury
    En 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...
  90. ncbi request reprint Gene expression of transferrin and transferrin receptor in brains of control vs. iron-deficient rats
    Jian 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...
  91. ncbi request reprint Redox metals in Alzheimer's disease
    Bozho 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...
  92. ncbi request reprint Ferritin levels in the cerebrospinal fluid and restless legs syndrome: effects of different clinical phenotypes
    Christopher J Earley
    Department of Neurology, Johns Hopkins School of Medicine, Johns Hopkins Bayview Medical Center, Baltimore, MD 21224, USA
    Sleep 28:1069-75. 2005
    ....
  93. ncbi request reprint Microscopic R2* mapping of reduced brain iron in the Belgrade rat
    Holly 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...
  94. ncbi request reprint Nitric oxide protects astrocytes from oxidative stress
    Sara 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...
  95. pmc Hypoxia-inducible factor prolyl 4-hydroxylase inhibition. A target for neuroprotection in the central nervous system
    Ambreena 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...
  96. ncbi request reprint Iron, brain ageing and neurodegenerative disorders
    Luigi 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...
  97. ncbi request reprint H and L ferritin subunit mRNA expression differs in brains of control and iron-deficient rats
    Jian 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...