Steven A Moore

Summary

Affiliation: University of Iowa
Country: USA

Publications

  1. ncbi request reprint Limb-girdle muscular dystrophies
    Federica Piccolo
    Department of Physiology and Biophysics and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    Adv Neurol 88:273-91. 2002
  2. doi request reprint Glial scaffold required for cerebellar granule cell migration is dependent on dystroglycan function as a receptor for basement membrane proteins
    Huy Nguyen
    Department of Pathology, 4270A Carver Biomedical Research Building, The University of Iowa, 285 Newton Road, Iowa City, IA 52242, USA
    Acta Neuropathol Commun 1:58. 2013
  3. pmc Variable disease severity in Saudi Arabian and Sudanese families with c.3924 + 2 T > C mutation of LAMA2
    Claudia Di Blasi
    Division of Neuromuscular Diseases and Neuroimmunology, Fondazione IRCCS Istituto Neurologico C, Besta, Milan, Italy
    BMC Res Notes 4:534. 2011
  4. ncbi request reprint Polyunsaturated fatty acid synthesis and release by brain-derived cells in vitro
    S A Moore
    Department of Pathology, The University of Iowa, Iowa City 52242, USA
    J Mol Neurosci 16:195-200; discussion 215-21. 2001
  5. ncbi request reprint Limb-girdle muscular dystrophy in the United States
    Steven A Moore
    University of Iowa, Iowa City, 52242, USA
    J Neuropathol Exp Neurol 65:995-1003. 2006
  6. pmc Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain
    Jakob S Satz
    Howard Hughes Medical Institute, and Department of Molecular Physiology, University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    J Neurosci 30:14560-72. 2010
  7. ncbi request reprint Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization
    Fumiaki Saito
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    Neuron 38:747-58. 2003
  8. ncbi request reprint Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy
    Steven A Moore
    Department of Pathology, The University of Iowa, Iowa City, Iowa 52242 1101, USA
    Nature 418:422-5. 2002
  9. pmc LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
    Matthew M Goddeeris
    1 Howard Hughes Medical Institute, Roy J and Lucille A Carver College of Medicine, The University of Iowa, Iowa City, Iowa 52242, USA 2 Department of Molecular Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, The University of Iowa, Iowa City, Iowa 52242, USA
    Nature 503:136-40. 2013
  10. ncbi request reprint LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies
    Rita Barresi
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA
    Nat Med 10:696-703. 2004

Research Grants

Detail Information

Publications46

  1. ncbi request reprint Limb-girdle muscular dystrophies
    Federica Piccolo
    Department of Physiology and Biophysics and Department of Neurology, Howard Hughes Medical Institute, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    Adv Neurol 88:273-91. 2002
  2. doi request reprint Glial scaffold required for cerebellar granule cell migration is dependent on dystroglycan function as a receptor for basement membrane proteins
    Huy Nguyen
    Department of Pathology, 4270A Carver Biomedical Research Building, The University of Iowa, 285 Newton Road, Iowa City, IA 52242, USA
    Acta Neuropathol Commun 1:58. 2013
    ..While cerebellar dysplasia is a common feature of dystroglycanopathy, its pathogenesis has not been thoroughly investigated...
  3. pmc Variable disease severity in Saudi Arabian and Sudanese families with c.3924 + 2 T > C mutation of LAMA2
    Claudia Di Blasi
    Division of Neuromuscular Diseases and Neuroimmunology, Fondazione IRCCS Istituto Neurologico C, Besta, Milan, Italy
    BMC Res Notes 4:534. 2011
    ..abstract:..
  4. ncbi request reprint Polyunsaturated fatty acid synthesis and release by brain-derived cells in vitro
    S A Moore
    Department of Pathology, The University of Iowa, Iowa City 52242, USA
    J Mol Neurosci 16:195-200; discussion 215-21. 2001
    ..These data suggest that endothelial cells and astrocytes cooperate in the local synthesis and release of PUFA, collectively maintaining a brain environment enriched in long-chain PUFA...
  5. ncbi request reprint Limb-girdle muscular dystrophy in the United States
    Steven A Moore
    University of Iowa, Iowa City, 52242, USA
    J Neuropathol Exp Neurol 65:995-1003. 2006
    ..The most common LGMDs in the United States are calpainopathies, dysferlinopathies, sarcoglycanopathies, and dystroglycanopathies...
  6. pmc Distinct functions of glial and neuronal dystroglycan in the developing and adult mouse brain
    Jakob S Satz
    Howard Hughes Medical Institute, and Department of Molecular Physiology, University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    J Neurosci 30:14560-72. 2010
    ..Differences in dystroglycan glycosylation in distinct cell types of the CNS may contribute to the diversity of dystroglycan function in the CNS, as well as to the broad clinical spectrum of type II lissencephalies...
  7. ncbi request reprint Unique role of dystroglycan in peripheral nerve myelination, nodal structure, and sodium channel stabilization
    Fumiaki Saito
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    Neuron 38:747-58. 2003
    ..Dystroglycan may be required for the normal maintenance of voltage-gated sodium channels at nodes of Ranvier, possibly by mediating trans interactions between Schwann cell microvilli and the nodal axolemma...
  8. ncbi request reprint Deletion of brain dystroglycan recapitulates aspects of congenital muscular dystrophy
    Steven A Moore
    Department of Pathology, The University of Iowa, Iowa City, Iowa 52242 1101, USA
    Nature 418:422-5. 2002
    ..Our data strongly support the hypothesis that defects in dystroglycan are central to the pathogenesis of structural and functional brain abnormalities seen in CMD...
  9. pmc LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy
    Matthew M Goddeeris
    1 Howard Hughes Medical Institute, Roy J and Lucille A Carver College of Medicine, The University of Iowa, Iowa City, Iowa 52242, USA 2 Department of Molecular Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, The University of Iowa, Iowa City, Iowa 52242, USA
    Nature 503:136-40. 2013
    ..Our results reveal that the LARGE-glycan of dystroglycan serves as a tunable extracellular matrix protein scaffold, the extension of which is required for normal skeletal muscle function. ..
  10. ncbi request reprint LARGE can functionally bypass alpha-dystroglycan glycosylation defects in distinct congenital muscular dystrophies
    Rita Barresi
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA
    Nat Med 10:696-703. 2004
    ..Our findings indicate that modulation of LARGE expression or activity is a viable therapeutic strategy for glycosyltransferase-deficient congenital muscular dystrophies...
  11. pmc Astrocytes in the rat nucleus tractus solitarii are critical for cardiovascular reflex control
    Li Hsien Lin
    Laboratory of Neurobiology, Department of Neurology, Carver College of Medicine, The University of Iowa and Iowa City Veterans Affairs Medical Center, Iowa City, Iowa 52242, and Department of Pathology, Caver College of Medicine, The University of Iowa, Iowa City, Iowa 52242
    J Neurosci 33:18608-17. 2013
    ..Thus, NTS astrocytes may play a role in mediating cardiovascular reflex transmission through the NTS. ..
  12. ncbi request reprint Induction of cyclooxygenase-2 by anandamide in cerebral microvascular endothelium
    Ping Chen
    Department of Biochemistry, 4 403 BSB, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA
    Microvasc Res 69:28-35. 2005
    ..These results indicate that AEA increased COX-2 expression at the transcriptional level through, at least in part, a cannabinoid receptor-1-mediated mechanism in cerebral microvascular endothelium...
  13. ncbi request reprint Disruption of DAG1 in differentiated skeletal muscle reveals a role for dystroglycan in muscle regeneration
    Ronald D Cohn
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, Department of Neurology, Iowa City, IA 52242, USA
    Cell 110:639-48. 2002
    ..Therefore, inadequate repair of skeletal muscle by satellite cells represents an important mechanism affecting the pathogenesis of muscular dystrophy...
  14. pmc Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan
    Renzhi Han
    Howard Hughes Medical Institute, Department of Molecular Physiology, The University of Iowa, Iowa City, IA 52242, USA
    Proc Natl Acad Sci U S A 106:12573-9. 2009
    ..These data provide direct mechanistic insight into how the dystroglycan-linked basal lamina contributes to the maintenance of sarcolemmal integrity and protects muscles from damage...
  15. pmc A common disease-associated missense mutation in alpha-sarcoglycan fails to cause muscular dystrophy in mice
    Kazuhiro Kobuke
    Howard Hughes Medical Institute, The University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    Hum Mol Genet 17:1201-13. 2008
    ..Our study presents an unexpected difference in the behavior of a missense-mutated protein in mice versus human patients, and emphasizes the need to understand species-specific protein quality control systems...
  16. pmc Brain and eye malformations resembling Walker-Warburg syndrome are recapitulated in mice by dystroglycan deletion in the epiblast
    Jakob S Satz
    Howard Hughes Medical Institute and Department of Molecular Physiology, University of Iowa, Iowa City, Iowa 52242, USA
    J Neurosci 28:10567-75. 2008
    ....
  17. pmc Visual impairment in the absence of dystroglycan
    Jakob S Satz
    Department of Molecular Physiology and Biophysics, Roy J and Lucille A Carver College of Medicine, Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa 52242, USA
    J Neurosci 29:13136-46. 2009
    ..In contrast to the role of alpha-dystroglycan extracellular interactions during early development of the CNS, beta-dystroglycan intracellular interactions are important for visual function but not the laminar development of the retina...
  18. pmc Sarcolemma-localized nNOS is required to maintain activity after mild exercise
    Yvonne M Kobayashi
    Howard Hughes Medical Institute, University of Iowa, Roy J and Lucille A Carver College of Medicine, 4283 Carver Biomedical Research Building, 285 Newton Road, Iowa City, Iowa 52242 1101, USA
    Nature 456:511-5. 2008
    ..Our results suggest that patients with an exaggerated fatigue response to mild exercise would show clinical improvement in response to treatment strategies aimed at improving exercise-induced signalling...
  19. ncbi request reprint Omega-oxidation of 20-hydroxyeicosatetraenoic acid (20-HETE) in cerebral microvascular smooth muscle and endothelium by alcohol dehydrogenase 4
    Xixuan H Collins
    Department of Biochemistry, Carver College of Medicine, The University of Iowa, Iowa City, Iowa 52242, USA
    J Biol Chem 280:33157-64. 2005
    ..Although ADH4 and ADH3 are expressed in MSMC and MEC, the inhibition produced by N-heptylformamide suggests that ADH4 is primarily responsible for 20-COOH-AA formation in the cerebral microvasculature...
  20. ncbi request reprint Sudden death and myocardial lesions after damage to catecholamine neurons of the nucleus tractus solitarii in rat
    William T Talman
    Department of Neurology, Carver College of Medicine, University of Iowa and Department of Veterans Affairs Medical Center, Iowa, IA 52242, USA
    Cell Mol Neurobiol 32:1119-26. 2012
    ..Thus, cardiac and cardiovascular effects of lesions directed toward catecholamine neurons of the NTS are similar to those following damage directed toward NK1 receptor-containing neurons...
  21. pmc ISPD loss-of-function mutations disrupt dystroglycan O-mannosylation and cause Walker-Warburg syndrome
    Tobias Willer
    Department of Molecular Physiology and Biophysics, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa, USA
    Nat Genet 44:575-80. 2012
    ..Finally, we show that recessive mutations in ISPD abolish the initial step in laminin-binding glycan synthesis by disrupting dystroglycan O-mannosylation. This establishes a new mechanism for WWS pathophysiology...
  22. pmc Elevated oxidative membrane damage associated with genetic modifiers of Lyst-mutant phenotypes
    Colleen M Trantow
    Department of Molecular Physiology and Biophysics, The University of Iowa, Iowa City, Iowa, United States of America
    PLoS Genet 6:e1001008. 2010
    ..These results identify an association between oxidative damage to lipid membranes and the severity of Lyst-mutant phenotypes, revealing a new mechanism that contributes to pathophysiology involving LYST...
  23. pmc Muscle fatigue increases the probability of developing hyperalgesia in mice
    Takeshi Yokoyama
    Physical Therapy and Rehabilitation Science Graduate Program, Department of Pathology, Pain Research Program, University of Iowa, Iowa City, Iowa 52242, USA
    J Pain 8:692-9. 2007
    ..Clinically, this could relate to the development of pain from such conditions as repetitive strain injury, and may relate to the interrelationship between chronic pain and fatigue...
  24. pmc Genetic ablation of complement C3 attenuates muscle pathology in dysferlin-deficient mice
    Renzhi Han
    Howard Hughes Medical Institute, Department of Molecular Physiology and Biophysics, Department of Neurology, Department of Internal Medicine, Roy J and Lucille A Carver College of Medicine, The University of Iowa, Iowa City, Iowa, USA
    J Clin Invest 120:4366-74. 2010
    ..These results demonstrate that complement-mediated muscle injury is central to the pathogenesis of dysferlinopathy and suggest that targeting the complement system might serve as a therapeutic approach for this disease...
  25. ncbi request reprint Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies
    Daniel E Michele
    Howard Hughes Medical Institute, Department of Physiology and Biophysics, University of Iowa, Iowa City, Iowa 52242 1101, USA
    Nature 418:417-22. 2002
    ....
  26. pmc Dystroglycan on radial glia end feet is required for pial basement membrane integrity and columnar organization of the developing cerebral cortex
    Timothy D Myshrall
    Department of Comparative Medicine, University of Washington, Seattle, Washington, USA
    J Neuropathol Exp Neurol 71:1047-63. 2012
    ....
  27. ncbi request reprint Limb-girdle muscular dystrophy
    Katherine D Mathews
    Departments of Pediatrics and Neurology, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 52242, USA
    Curr Neurol Neurosci Rep 3:78-85. 2003
    ..For example, LGMD 2H appears to be due to mutations affecting the ubiquitin-proteasome pathway. A diagnostic approach is outlined based on clinical features, genetics, and commercially available testing...
  28. ncbi request reprint Hughlings Jackson and the role of the entorhinal cortex in temporal lobe epilepsy: from patient A to Doctor Z
    Sahib S Khalsa
    Department of Neurology, 1216 MERF, University of Iowa, Iowa City, IA 52242, USA
    Epilepsy Behav 9:524-31. 2006
    ..This finding validates Jackson's original description and suggests that the entorhinal cortex can play a role in seizure genesis...
  29. ncbi request reprint Cyclooxygenase-2 expression and activity are induced by amyloid-beta 1-42 in brain derived cells
    MacKenzie Hilfers Bilunas
    Department of Pathology, University of Iowa, 1046 Medical Laboratories, Iowa City, IA 52242, USA
    Adv Exp Med Biol 507:93-9. 2002
  30. pmc Cardiac pathology exceeds skeletal muscle pathology in two cases of limb-girdle muscular dystrophy type 2I
    Marta Margeta
    Department of Pathology, University of California San Francisco, 513 Parnassus Avenue, HSW 514, San Francisco, California 94143, USA
    Muscle Nerve 40:883-9. 2009
    ....
  31. pmc LMNA variants cause cytoplasmic distribution of nuclear pore proteins in Drosophila and human muscle
    George Dialynas
    Department of Biochemistry, University of Iowa, Iowa City, IA 52242, USA
    Hum Mol Genet 21:1544-56. 2012
    ....
  32. doi request reprint Evaluation of commercial dysferlin antibodies on canine, mouse and human skeletal muscle
    Ling T Guo
    Department of Pathology, University of California San Diego, La Jolla, CA, USA
    Neuromuscul Disord 20:820-5. 2010
    ..Use of select antibodies for immunostaining and immunoblotting, and optimization of immunostaining methods, should increase the sensitivity of detecting dysferlin deficiency in skeletal muscle...
  33. ncbi request reprint Identification of a functional peroxisome proliferator-activated receptor response element in the rat catalase promoter
    Geoffrey D Girnun
    Free Radical and Radiation Biology Program, University of Iowa, Iowa City, IA 52242, USA
    Mol Endocrinol 16:2793-801. 2002
    ..Thus, in addition to transrepression of proinflammatory transcription factors, PPARgamma may also be modulating catalase expression, and hence down-regulating the inflammatory response via scavenging of reactive oxygen species...
  34. ncbi request reprint Metabolism of anandamide in cerebral microvascular endothelial cells
    Ping Chen
    Department of Biochemistry, University of Iowa College of Medicine, 4 403 BSB, Iowa City, IA 52242, USA
    Prostaglandins Other Lipid Mediat 73:59-72. 2004
    ....
  35. pmc Unilateral carrageenan injection into muscle or joint induces chronic bilateral hyperalgesia in rats
    Rajan Radhakrishnan
    Graduate Program in Physical Therapy and Rehabilitation Science, University of Iowa, 100 Medical Education Building 1 252, Iowa, IA 52242 1190, USA
    Pain 104:567-77. 2003
    ..Furthermore, 3% carrageenan injected into deep tissues produces hyperalgesia that spreads to the contralateral side, at the same time period as the inflammation transforms from acute to chronic...
  36. pmc CaMKII determines mitochondrial stress responses in heart
    Mei ling A Joiner
    Department of Internal Medicine and Cardiovascular Center, Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA
    Nature 491:269-73. 2012
    ....
  37. pmc Infantile onset CMT2D/dSMA V in monozygotic twins due to a mutation in the anticodon-binding domain of GARS
    Jamie M Eskuri
    The University of Iowa Carver College of Medicine, 200 Hawkins Drive, Iowa City, IA 52242, USA
    J Peripher Nerv Syst 17:132-4. 2012
    ....
  38. ncbi request reprint The oxygen radical scavenger pyrrolidine dithiocarbamate enhances interleukin-1beta-induced cyclooxygenase-2 expression in cerebral microvascular smooth muscle cells
    Xiang Fang
    Department of Biochemistry, University of Iowa College of Medicine, Iowa City, IA 52242, USA
    Microvasc Res 64:405-13. 2002
    ..These results suggest that endogenous oxygen radicals may blunt COX-2 induced by IL-1beta through an effect on translation...
  39. pmc Cardiac damage after lesions of the nucleus tractus solitarii
    Ameya Nayate
    Department of Neurology, University of Iowa, Iowa City, IA 52242, USA
    Am J Physiol Regul Integr Comp Physiol 296:R272-9. 2009
    ..This study supports the hypothesis that NTS lesions interrupting the baroreflex may induce cardiac arrhythmias and myocardial changes similar to those seen in humans with central lesions and may lead to sudden cardiac death...
  40. doi request reprint Dysferlin-deficient muscular dystrophy features amyloidosis
    Simone Spuler
    Muscle Research Unit, Experimental and Clinical Research Center at the Charité and the Max Delbrück Center, Berlin, Germany
    Ann Neurol 63:323-8. 2008
    ..Dysferlin (DYSF) gene mutations cause limb girdle muscular dystrophy type 2B and Miyoshi's myopathy. The consequences of DYSF mutations on protein structure are poorly understood...
  41. pmc Collagen VI glycine mutations: perturbed assembly and a spectrum of clinical severity
    Rishika A Pace
    Murdoch Childrens Research Institute and Department of Paediatrics, University of Melbourne, Royal Children s Hospital, Victoria, Australia
    Ann Neurol 64:294-303. 2008
    ..Glycine mutations in the triple helix have been identified in both Bethlem and Ullrich congenital muscular dystrophy, but it is not known why they cause these different phenotypes...
  42. ncbi request reprint Human angiotensin II type-2 receptor inhibition of insulin-mediated ERK-2 activity via a G-protein coupled signaling pathway
    Steven A Moore
    Department of Cell and Structural Biology, University of Illinois, B107 Chemical and Life Science Building, 601 S Goodwin Ave, Urbana, IL 61801, USA
    Brain Res Mol Brain Res 124:62-9. 2004
    ..Our results support the hypothesis that the AT(2) receptor inhibits insulin-induced ERK-2 activity via a G-protein coupled pathway involving the up-regulation of PP2A...
  43. ncbi request reprint Deep white matter pathologic features in watershed regions: a novel pattern of central nervous system involvement in MELAS
    Liana G Apostolova
    Department of Neurology, University of California, Los Angeles, USA
    Arch Neurol 62:1154-6. 2005
    ..Magnetic resonance imaging (MRI) abnormalities typically involve the cortical gray and the adjacent subcortical white matter...
  44. pmc Both laminin and Schwann cell dystroglycan are necessary for proper clustering of sodium channels at nodes of Ranvier
    Simona Occhi
    DIBIT, San Raffaele Scientific Institute, 20132 Milan, Italy
    J Neurosci 25:9418-27. 2005
    ..Finally, abnormal sodium channel clusters are present in a patient with MDC1A, providing a molecular basis for the reduced nerve conduction velocity in this disorder...
  45. pmc Progressive external ophthalmoplegia and vision and hearing loss in a patient with mutations in POLG2 and OPA1
    Silvio Ferraris
    Department of Pediatrics, University of Turin, Turin, Italy
    Arch Neurol 65:125-31. 2008
    ..To describe the clinical features, muscle pathological characteristics, and molecular studies of a patient with a mutation in the gene encoding the accessory subunit (p55) of polymerase gamma (POLG2) and a mutation in the OPA1 gene...
  46. ncbi request reprint 20-Hydroxyeicosatetraenoic acid is a potent dilator of mouse basilar artery: role of cyclooxygenase
    Xiang Fang
    Dept of Medicine, Harbor Hospital Center, 3001 S Hanover St, Baltimore MD 21225, USA
    Am J Physiol Heart Circ Physiol 291:H2301-7. 2006
    ..Whereas the conversion of 20-HETE to 20-OH-PGE(2) and production of endogenous prostaglandins probably are primarily responsible for vasodilation, the production of 20-COOH-AA also may contribute to this process...

Research Grants2

  1. Astrocyte dystroglycan complexes in brain development
    Steven Moore; Fiscal Year: 2003
    ..This strategy should disrupt the astrocyte DGC beginning in the latter half of embryonic development. We believe this strategy will produce mice with neuronal migration and cerebrovascular defects. ..