spectrin

Summary

Summary: A high molecular weight (220-250 kDa) water-soluble protein which can be extracted from erythrocyte ghosts in low ionic strength buffers. The protein contains no lipids or carbohydrates, is the predominant species of peripheral erythrocyte membrane proteins, and exists as a fibrous coating on the inner, cytoplasmic surface of the membrane.

Top Publications

  1. ncbi Spectrin and ankyrin-based pathways: metazoan inventions for integrating cells into tissues
    V Bennett
    Howard Hughes Medical Institute, Duke University Medical Center, Durham, North Carolina 27710, USA
    Physiol Rev 81:1353-92. 2001
  2. pmc Actin, spectrin, and associated proteins form a periodic cytoskeletal structure in axons
    Ke Xu
    Howard Hughes Medical Institute, Department of Chemistry and Chemical Biology, Harvard University, Cambridge, MA 02138, USA
    Science 339:452-6. 2013
  3. pmc Loss of beta-III spectrin leads to Purkinje cell dysfunction recapitulating the behavior and neuropathology of spinocerebellar ataxia type 5 in humans
    Emma M Perkins
    The Centre for Integrative Physiology, The University of Edinburgh, George Square, Edinburgh EH8 9XD, United Kingdom
    J Neurosci 30:4857-67. 2010
  4. ncbi Crumbs, the Drosophila homologue of human CRB1/RP12, is essential for photoreceptor morphogenesis
    Milena Pellikka
    Department of Zoology, University of Toronto, Toronto, Ontario M5S 3G5, Canada
    Nature 416:143-9. 2002
  5. ncbi The spectrin repeat: a structural platform for cytoskeletal protein assemblies
    Kristina Djinovic-Carugo
    Structural Biology Laboratory, Sincrotrone Trieste in AREA Science Park, S S 14 Km 163, 5, 34012 Trieste, Italy
    FEBS Lett 513:119-23. 2002
  6. pmc A postsynaptic spectrin scaffold defines active zone size, spacing, and efficacy at the Drosophila neuromuscular junction
    Jan Pielage
    Department of Biochemistry and Biophysics, University of California, San Francisco, San Francisco, CA 94143, USA
    J Cell Biol 175:491-503. 2006
  7. ncbi Plectin and IFAP-300K are homologous proteins binding to microtubule-associated proteins 1 and 2 and to the 240-kilodalton subunit of spectrin
    H Herrmann
    J Biol Chem 262:1320-5. 1987
  8. ncbi Nesprins: a novel family of spectrin-repeat-containing proteins that localize to the nuclear membrane in multiple tissues
    Q Zhang
    Department of Medicine, Division of Cardiovascular Medicine, University of Cambridge, Box 110, Addenbrooke s Hospital, Hills Road, Cambridge, CB2 2QQ, UK
    J Cell Sci 114:4485-98. 2001
  9. pmc Spectrin-based skeleton as an actor in cell signaling
    B Machnicka
    University of Zielona Gora, Zielona Gora, Poland
    Cell Mol Life Sci 69:191-201. 2012
  10. pmc Control of erythrocyte membrane-skeletal cohesion by the spectrin-membrane linkage
    Lionel Blanc
    Red Cell Physiology Laboratory, New York Blood Center, New York, New York 10065, USA
    Biochemistry 49:4516-23. 2010

Detail Information

Publications308 found, 100 shown here

  1. ncbi Spectrin and ankyrin-based pathways: metazoan inventions for integrating cells into tissues
    V Bennett
    Howard Hughes Medical Institute, Duke University Medical Center, Durham, North Carolina 27710, USA
    Physiol Rev 81:1353-92. 2001
    The spectrin-based membrane skeleton of the humble mammalian erythrocyte has provided biologists with a set of interacting proteins with diverse roles in organization and survival of cells in metazoan organisms...
  2. pmc Actin, spectrin, and associated proteins form a periodic cytoskeletal structure in axons
    Ke Xu
    Howard Hughes Medical Institute, Department of Chemistry and Chemical Biology, Harvard University, Cambridge, MA 02138, USA
    Science 339:452-6. 2013
    Actin and spectrin play important roles in neurons, but their organization in axons and dendrites remains unclear...
  3. pmc Loss of beta-III spectrin leads to Purkinje cell dysfunction recapitulating the behavior and neuropathology of spinocerebellar ataxia type 5 in humans
    Emma M Perkins
    The Centre for Integrative Physiology, The University of Edinburgh, George Square, Edinburgh EH8 9XD, United Kingdom
    J Neurosci 30:4857-67. 2010
    Mutations in SPTBN2, the gene encoding beta-III spectrin, cause spinocerebellar ataxia type 5 in humans (SCA5), a neurodegenerative disorder resulting in loss of motor coordination...
  4. ncbi Crumbs, the Drosophila homologue of human CRB1/RP12, is essential for photoreceptor morphogenesis
    Milena Pellikka
    Department of Zoology, University of Toronto, Toronto, Ontario M5S 3G5, Canada
    Nature 416:143-9. 2002
    ..Crumbs also regulates stalk development by stabilizing the membrane-associated spectrin cytoskeleton, a function mechanistically distinct from its role in epithelial apical-basal polarity...
  5. ncbi The spectrin repeat: a structural platform for cytoskeletal protein assemblies
    Kristina Djinovic-Carugo
    Structural Biology Laboratory, Sincrotrone Trieste in AREA Science Park, S S 14 Km 163, 5, 34012 Trieste, Italy
    FEBS Lett 513:119-23. 2002
    b>Spectrin repeats are three-helix bundle structures which occur in a large number of diverse proteins, either as single copies or in tandem arrangements of multiple repeats...
  6. pmc A postsynaptic spectrin scaffold defines active zone size, spacing, and efficacy at the Drosophila neuromuscular junction
    Jan Pielage
    Department of Biochemistry and Biophysics, University of California, San Francisco, San Francisco, CA 94143, USA
    J Cell Biol 175:491-503. 2006
    ..In this study, we document a role for the postsynaptic Spectrin skeleton in this process...
  7. ncbi Plectin and IFAP-300K are homologous proteins binding to microtubule-associated proteins 1 and 2 and to the 240-kilodalton subunit of spectrin
    H Herrmann
    J Biol Chem 262:1320-5. 1987
    ..plectin bound to vimentin, microtubule-associated proteins 1 and 2, the 240-kDa chain of brain fodrin, and alpha-spectrin from human erythrocytes...
  8. ncbi Nesprins: a novel family of spectrin-repeat-containing proteins that localize to the nuclear membrane in multiple tissues
    Q Zhang
    Department of Medicine, Division of Cardiovascular Medicine, University of Cambridge, Box 110, Addenbrooke s Hospital, Hills Road, Cambridge, CB2 2QQ, UK
    J Cell Sci 114:4485-98. 2001
    ..We have named these proteins nesprin-1 and -2 for nuclear envelope spectrin repeat, as they are characterized by the presence of multiple, clustered spectrin repeats, bipartite nuclear ..
  9. pmc Spectrin-based skeleton as an actor in cell signaling
    B Machnicka
    University of Zielona Gora, Zielona Gora, Poland
    Cell Mol Life Sci 69:191-201. 2012
    ..We discuss new data concerning the commonly known role of the spectrin-based skeleton in control of membrane organization, stability and shape, and tethering protein mosaics to the ..
  10. pmc Control of erythrocyte membrane-skeletal cohesion by the spectrin-membrane linkage
    Lionel Blanc
    Red Cell Physiology Laboratory, New York Blood Center, New York, New York 10065, USA
    Biochemistry 49:4516-23. 2010
    b>Spectrin tetramer is the major structural member of the membrane-associated skeletal network of red cells...
  11. pmc Recessive mutations in SPTBN2 implicate β-III spectrin in both cognitive and motor development
    Stefano Lise
    Wellcome Trust Centre for Human Genetics, University of Oxford, Oxford, United Kingdom
    PLoS Genet 8:e1003074. 2012
    III spectrin is present in the brain and is known to be important in the function of the cerebellum...
  12. pmc Beta-III spectrin mutation L253P associated with spinocerebellar ataxia type 5 interferes with binding to Arp1 and protein trafficking from the Golgi
    Yvonne L Clarkson
    The Centre for Integrative Physiology, The University of Edinburgh, Edinburgh, UK
    Hum Mol Genet 19:3634-41. 2010
    ..ataxia type 5 (SCA5) is an autosomal dominant neurodegenerative disorder caused by mutations in beta-III spectrin. A mouse lacking full-length beta-III spectrin has a phenotype closely mirroring symptoms of SCA5 patients...
  13. pmc alphaII-Spectrin breakdown product cerebrospinal fluid exposure metrics suggest differences in cellular injury mechanisms after severe traumatic brain injury
    Gretchen M Brophy
    Departments of Pharmacy and Neurosurgery, Virginia Commonwealth University, Medical College of Virginia Campus, Richmond, Virginia 23298 0533, USA
    J Neurotrauma 26:471-9. 2009
    Traumatic brain injury (TBI) produces alphaII-spectrin breakdown products (SBDPs) that are potential biomarkers for TBI...
  14. ncbi Spectrin organization and dynamics: new insights
    Abhijit Chakrabarti
    Biophysics Division, Saha Institute of Nuclear Physics, Kolkata 700 064, India
    Biosci Rep 26:369-86. 2006
    b>Spectrin is the major constituent protein of the erythrocyte cytoskeleton which forms a filamentous network on the cytoplasmic face of the membrane by providing a scaffold for a variety of proteins...
  15. ncbi Kalirin, a cytosolic protein with spectrin-like and GDP/GTP exchange factor-like domains that interacts with peptidylglycine alpha-amidating monooxygenase, an integral membrane peptide-processing enzyme
    M R Alam
    Departments of Neuroscience and Physiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Biol Chem 272:12667-75. 1997
    ..P-CIP10 is a 217-kDa cytosolic protein with nine spectrin-like repeats and adjacent Dbl homology and pleckstrin homology domains typical of GDP/GTP exchange factors...
  16. pmc Structural basis for spectrin recognition by ankyrin
    Jonathan J Ipsaro
    Department of Biochemistry, Molecular Biology, and Cell Biology, Northwestern University, Evanston, IL 60208, USA
    Blood 115:4093-101. 2010
    ..b>Spectrin, the principal component of this network, is anchored to membrane proteins through the adaptor protein ankyrin...
  17. ncbi Presynaptic spectrin is essential for synapse stabilization
    Jan Pielage
    Department of Biochemistry and Biophysics, Program in Neuroscience, University of California, San Francisco, San Francisco, California 94143, USA
    Curr Biol 15:918-28. 2005
    ..Precise neural circuitry is established and maintained through a regulated balance of synapse stabilization and disassembly. Currently, little is known about the molecular mechanisms that specify synapse stability versus disassembly...
  18. ncbi Spectrin mutations cause spinocerebellar ataxia type 5
    Yoshio Ikeda
    Department of Genetics, Cell Biology, and Development, University of Minnesota, 321 Church St SE, Minneapolis, Minnesota 55455 USA
    Nat Genet 38:184-90. 2006
    We have discovered that beta-III spectrin (SPTBN2) mutations cause spinocerebellar ataxia type 5 (SCA5) in an 11-generation American kindred descended from President Lincoln's grandparents and two additional families...
  19. ncbi Organizing the fluid membrane bilayer: diseases linked to spectrin and ankyrin
    Vann Bennett
    Howard Hughes Medical Institute, Duke University Medical Center, Durham, NC 27710, USA
    Trends Mol Med 14:28-36. 2008
    Ankyrin and spectrin were first discovered as binding partners in the membrane skeleton of human erythrocytes. Mutations in genes encoding these proteins cause hereditary spherocytosis...
  20. pmc Membrane domains based on ankyrin and spectrin associated with cell-cell interactions
    Vann Bennett
    Howard Hughes Medical Institute, and Department of Cell Biology, Duke University Medical Center, Durham, North Carolina 27710, USA
    Cold Spring Harb Perspect Biol 1:a003012. 2009
    ..these domains all share on their cytoplasmic surfaces a membrane skeleton comprised of members of the ankyrin and spectrin families...
  21. pmc Axons break in animals lacking beta-spectrin
    Marc Hammarlund
    Department of Biology, Howard Hughes Medical Institute, University of Utah, Salt Lake City, UT 84112, USA
    J Cell Biol 176:269-75. 2007
    ..In this study, we demonstrate that beta-spectrin is required for the physical integrity of neuronal processes in the nematode Caenorhabditis elegans...
  22. ncbi Nonerythroid alphaII spectrin is required for recruitment of FANCA and XPF to nuclear foci induced by DNA interstrand cross-links
    Deepa Sridharan
    Department of Pathology and Laboratory Medicine, UMDNJ New Jersey Medical School and the Graduate School of Biomedical Sciences, Newark, NJ 07103, USA
    J Cell Sci 116:823-35. 2003
    ..The present study demonstrates that the structural protein nonerythroid alpha spectrin (alphaSpIISigma*), present in normal human cell nuclei, plays an important role in repair of DNA interstrand ..
  23. pmc Crumbs interacts with moesin and beta(Heavy)-spectrin in the apical membrane skeleton of Drosophila
    Emmanuelle Médina
    Laboratoire de Neurogenèse et Morphogenèse dans le Développement et l Adulte, Institut de Biologie du Developpement de Marseille, Universite de la Mediterranee, 13288 Marseille, Cedex 09, France
    J Cell Biol 158:941-51. 2002
    ..The apical spectrin-based membrane skeleton (SBMS) is a protein network that is essential for epithelial morphogenesis and ZA ..
  24. pmc αII-spectrin breakdown products (SBDPs): diagnosis and outcome in severe traumatic brain injury patients
    Stefania Mondello
    Department of Clinical Programs and Center of Innovative Research, University of Florida, Gainesville, Florida, USA
    J Neurotrauma 27:1203-13. 2010
    In this study we assessed the clinical utility of quantitative assessments of alphaII-spectrin breakdown products (SBDP145 produced by calpain, and SBDP120 produced by caspase-3) in cerebrospinal fluid (CSF) as markers of brain damage and ..
  25. pmc Dystrophins carrying spectrin-like repeats 16 and 17 anchor nNOS to the sarcolemma and enhance exercise performance in a mouse model of muscular dystrophy
    Yi Lai
    Department of Molecular Microbiology and Immunology, University of Missouri, One Hospital Drive, Columbia, MO 65212, USA
    J Clin Invest 119:624-35. 2009
    ..transfection of dystrophin deletion constructs, we show that sarcolemmal targeting of nNOS was dependent on the spectrin-like repeats 16 and 17 (R16/17) within the rod domain...
  26. pmc Targeted deletion of betaIII spectrin impairs synaptogenesis and generates ataxic and seizure phenotypes
    Michael C Stankewich
    Department of Pathology, Yale University, New Haven, CT 06520, USA
    Proc Natl Acad Sci U S A 107:6022-7. 2010
    The spectrin membrane skeleton controls the disposition of selected membrane channels, receptors, and transporters...
  27. ncbi Effect of radiographic contrast media (Iodixanol, Iopromide) on the spectrin/actin-network of the membranous cytoskeleton of erythrocytes
    R P Franke
    University of Ulm, ZBMT, Department of Biomaterials, Ulm, Germany
    Clin Hemorheol Microcirc 54:273-85. 2013
    ..The shape of the spectrin net was completely altered; from the more homogeneous distribution--typical of cells in autologous plasma and ..
  28. ncbi The molecular basis of hereditary red cell membrane disorders
    Jean Delaunay
    AP HP, Hopital de Bicetre, Service d Hematologie, INSERM Unité 779, Faculte de Medecine Paris Sud, Le Kremlin Bicetre, France
    Blood Rev 21:1-20. 2007
    ..be underscored: widespread weak alleles modulate the expression of the SPTA1 gene, encoding the alpha-chain of spectrin; mutations in the anion exchanger can give rise to an array of distinct nosological entities, including a renal ..
  29. ncbi The CKK domain (DUF1781) binds microtubules and defines the CAMSAP/ssp4 family of animal proteins
    Anthony J Baines
    Department of Biosciences, University of Kent, Canterbury, Kent, United Kingdom
    Mol Biol Evol 26:2005-14. 2009
    We describe a structural domain common to proteins related to human calmodulin-regulated spectrin-associated protein1 (CAMSAP1)...
  30. pmc The transitional junction: a new functional subcellular domain at the intercalated disc
    Pauline M Bennett
    Randall Division of Cell and Molecular Biophysics, GKT School of Biomedical Sciences, King s College London, Guy s Campus, London SE1 1UL, United Kingdom
    Mol Biol Cell 17:2091-100. 2006
    ..Immunofluorescence and immunogold electron microscopy reveal a spectrin-rich domain at the apex of the folds...
  31. pmc Reductions of erythrocyte membrane viscoelastic coefficients reflect spectrin deficiencies in hereditary spherocytosis
    R E Waugh
    Department of Biophysics, University of Rochester School of Medicine and Dentistry, New York 14642
    J Clin Invest 81:133-41. 1988
    Hereditary spherocytosis is a common hemolytic anemia associated with deficiencies in spectrin, the principal structural protein of the erythrocyte membrane-skeleton...
  32. ncbi The spectrin-ankyrin-4.1-adducin membrane skeleton: adapting eukaryotic cells to the demands of animal life
    Anthony J Baines
    School of Biosciences and Centre for Biomedical Informatics, University of Kent, Canterbury, CT2 7NJ, UK
    Protoplasma 244:99-131. 2010
    ..These and other adaptations to animal life seem to require four proteins--spectrin, ankyrin, 4.1 and adducin--which emerged during eumetazoan evolution...
  33. pmc Cell organization, growth, and neural and cardiac development require αII-spectrin
    Michael C Stankewich
    Department of Pathology, Yale University School of Medicine, 310 Cedar St BML 150, New Haven, CT 06520, USA
    J Cell Sci 124:3956-66. 2011
    b>Spectrin α2 (αII-spectrin) is a scaffolding protein encoded by the Spna2 gene and constitutively expressed in most tissues. Exon trapping of Spna2 in C57BL/6 mice allowed targeted disruption of αII-spectrin...
  34. ncbi alphaII-betaV spectrin bridges the plasma membrane and cortical lattice in the lateral wall of the auditory outer hair cells
    Kirian Legendre
    Institut Pasteur, Unite de Genetique et Physiologie de l Audition, 25 rue du Dr Roux, 75015 Paris, France
    J Cell Sci 121:3347-56. 2008
    ..lattice, the highly organized cytoskeleton underlying the OHC lateral plasma membrane, is made up of F-actin and spectrin. Here, we show that alphaII and two of the five beta-spectrin subunits, betaII and betaV, are present in OHCs...
  35. pmc Identification of a small cytoplasmic ankyrin (AnkG119) in the kidney and muscle that binds beta I sigma spectrin and associates with the Golgi apparatus
    P Devarajan
    Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 06520, USA
    J Cell Biol 133:819-30. 1996
    ..from the human kidney which lacks part of this repetitive domain and associates in MDCK cells with beta I sigma spectrin and the Golgi apparatus, but not the plasma membrane...
  36. pmc Crystal structure of a rigid four-spectrin-repeat fragment of the human desmoplakin plakin domain
    Hee Jung Choi
    Department of Structural Biology, Stanford University School of Medicine, Stanford, CA 94305, USA
    J Mol Biol 409:800-12. 2011
    ..Plakin domains contain multiple copies of spectrin repeats (SRs)...
  37. pmc Na,K-ATPase transport from endoplasmic reticulum to Golgi requires the Golgi spectrin-ankyrin G119 skeleton in Madin Darby canine kidney cells
    P Devarajan
    Department of Pediatrics, Yale University, New Haven, CT 06520, USA
    Proc Natl Acad Sci U S A 94:10711-6. 1997
    b>Spectrin (betaISigma*) and ankyrin (AnkG119) associate with Golgi membranes and the dynactin complex, but their role in vesicle trafficking remains uncertain...
  38. pmc β-III spectrin is critical for development of purkinje cell dendritic tree and spine morphogenesis
    Yuanzheng Gao
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, Maryland 21205, USA
    J Neurosci 31:16581-90. 2011
    Mutations in the gene encoding β-III spectrin give rise to spinocerebellar ataxia type 5, a neurodegenerative disease characterized by progressive thinning of the molecular layer, loss of Purkinje cells and increasing motor deficits...
  39. ncbi A novel marker for traumatic brain injury: CSF alphaII-spectrin breakdown product levels
    N C Ringger
    Department of Neuroscience, Evelyn F and William L McKnight Brain Institute, University of Florida, Gainesville, Florida 32610, USA
    J Neurotrauma 21:1443-56. 2004
    ..Calpain cleaves alphaII-spectrin into breakdown products (SBDP) after TBI and ischemia...
  40. pmc Molecular mechanics of the alpha-actinin rod domain: bending, torsional, and extensional behavior
    Javad Golji
    Molecular Cell Biomechanics Laboratory, University of California Berkeley, Berkeley, California, USA
    PLoS Comput Biol 5:e1000389. 2009
    ..extend under mechanical stress, yet has a highly rigid interior region stabilized by aromatic packing within each spectrin repeat, by electrostatic interactions between the spectrin repeats, and by strong salt bridges between its two ..
  41. pmc A distal axonal cytoskeleton forms an intra-axonal boundary that controls axon initial segment assembly
    Mauricio R Galiano
    Department of Neuroscience, Baylor College of Medicine, Houston, TX 77030, USA
    Cell 149:1125-39. 2012
    ..break symmetry, they assemble a distal axonal submembranous cytoskeleton, comprised of ankyrinB (ankB), αII-spectrin, and βII-spectrin, that defines a boundary limiting ankG to the proximal axon...
  42. pmc Essential control of an endothelial cell ISOC by the spectrin membrane skeleton
    S Wu
    Department of Pharmacology, College of Medicine, University of South Alabama, Mobile, AL 36688, USA
    J Cell Biol 154:1225-33. 2001
    ..We examined whether the spectrin membrane skeleton couples Ca2+ store depletion to Ca2+ entry...
  43. ncbi Crystal structure of the repetitive segments of spectrin
    Y Yan
    Department of Biochemistry, Harvard University, Cambridge, MA 02138
    Science 262:2027-30. 1993
    The elongated proteins of the spectrin family (dystrophin, alpha-actinin, and spectrin) contain tandemly repeated segments and form resilient cellular meshworks by cross-linking actin filaments...
  44. ncbi The 8th and 9th tandem spectrin-like repeats of utrophin cooperatively form a functional unit to interact with polarity-regulating kinase PAR-1b
    Kazunari Yamashita
    Department of Molecular Biology, Yokohama City University Graduate School of Medical Science, 3 9 Fuku ura, Kanazawa Ku, Yokohama 236 0004, Japan
    Biochem Biophys Res Commun 391:812-7. 2010
    ..Utrophin is a large spectrin-like protein whose C-terminal domain mediates anchorage to a laminin receptor, dystroglycan (DG)...
  45. ncbi Evolution of spectrin function in cytoskeletal and membrane networks
    Anthony J Baines
    Department of Biosciences and Centre for Biomedical Informatics, University of Kent, Canterbury, Kent CT2 7NJ, UK
    Biochem Soc Trans 37:796-803. 2009
    b>Spectrin is a cytoskeletal protein thought to have descended from an alpha-actinin-like ancestor...
  46. ncbi Proteolysis of neuronal cytoskeletal proteins by calpain contributes to rat retinal cell death induced by hypoxia
    Yoshiyuki Tamada
    Senju Laboratory of Ocular Sciences, Senju Pharmaceutical Corporation Limited, Beaverton, OR 97006, USA
    Brain Res 1050:148-55. 2005
    ..This conclusion was based on data showing proteolysis of a calpain substrate alpha-spectrin, autolysis of activated calpain, and reduction of cell damage by calpain inhibitor SJA6017...
  47. pmc Structures of the spectrin-ankyrin interaction binding domains
    Jonathan J Ipsaro
    Department of Biochemistry, Molecular Biology, and Cell Biology, Northwestern University, Evanston, IL 60208, USA
    Blood 113:5385-93. 2009
    As key components of the erythrocyte membrane skeleton, spectrin and ankyrin specifically interact to tether the spectrin cytoskeleton to the cell membrane...
  48. ncbi Identification of the spectrin subunit and domains required for formation of spectrin/adducin/actin complexes
    X Li
    Department of Cell Biology, Duke University Medical Center, Durham, North Carolina 27710, USA
    J Biol Chem 271:15695-702. 1996
    Adducin is an actin-binding protein that has been proposed to function as a regulated assembly factor for the spectrin/actin network...
  49. pmc Ankyrin-G coordinates assembly of the spectrin-based membrane skeleton, voltage-gated sodium channels, and L1 CAMs at Purkinje neuron initial segments
    S M Jenkins
    Howard Hughes Medical Institute and Department of Biochemistry, Duke University Medical Center, Durham, NC 27710, USA
    J Cell Biol 155:739-46. 2001
    ..6, betaIV spectrin, and the L1 cell adhesion molecules (L1 CAMs) neurofascin and NrCAM at initial segments of cerebellar Purkinje ..
  50. pmc A β(IV)-spectrin/CaMKII signaling complex is essential for membrane excitability in mice
    Thomas J Hund
    Department of Internal Medicine, Division of Cardiovascular Medicine, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242, USA
    J Clin Invest 120:3508-19. 2010
    ..Here, we have identified β(IV)-spectrin as a multifunctional regulatory platform for Na(+) channels in mice...
  51. pmc betaIV spectrin, a new spectrin localized at axon initial segments and nodes of ranvier in the central and peripheral nervous system
    S Berghs
    Department of Internal Medicine, Section of Endocrinology, Yale University School of Medicine, New Haven, Connecticut 06510, USA
    J Cell Biol 151:985-1002. 2000
    We report the identification of betaIV spectrin, a novel spectrin isolated as an interactor of the receptor tyrosine phosphatase-like protein ICA512. The betaIV spectrin gene is located on human and mouse chromosomes 19q13...
  52. ncbi Spectrin and calpain: a 'target' and a 'sniper' in the pathology of neuronal cells
    A Czogalla
    Institute of Biochemistry and Molecular Biology, University of Wrocław, ul Przybyszewskiego 63 77, 51 148, Wrocław, Poland
    Cell Mol Life Sci 62:1913-24. 2005
    ..Calpain-mediated proteolysis of major membrane-skeletal protein, alphaII-spectrin, results in the appearance of two unique and highly stable breakdown products, which is an early event in neural ..
  53. pmc Experimental evidence for a frustrated energy landscape in a three-helix-bundle protein family
    Beth G Wensley
    Department of Chemistry, University of Cambridge, MRC Centre for Protein Engineering, Lensfield Rd, Cambridge CB2 1EW UK
    Nature 463:685-8. 2010
    ..Such general observations cannot explain the folding behaviour of the R15, R16 and R17 domains of alpha-spectrin. R15 folds approximately 3,000 times faster than its homologues, although they have similar structures, ..
  54. pmc Dominant-negative mutations in alpha-II spectrin cause West syndrome with severe cerebral hypomyelination, spastic quadriplegia, and developmental delay
    Hirotomo Saitsu
    Department of Human Genetics, Yokohama City University Graduate School of Medicine, 3 9 Fukuura, Kanazawa Ku, Yokohama 236 0004, Japan
    Am J Hum Genet 86:881-91. 2010
    ..SPTAN1 encoding alpha-II spectrin, which is essential for proper myelination in zebrafish, turned out to be deleted...
  55. pmc Spectrin mutations that cause spinocerebellar ataxia type 5 impair axonal transport and induce neurodegeneration in Drosophila
    DAMARIS N LORENZO
    Department of Genetics, Cell Biology, and Development, University of Minnesota, Minneapolis, MN 55455, USA
    J Cell Biol 189:143-58. 2010
    ..is an autosomal dominant neurodegenerative disorder caused by mutations in the SPBTN2 gene encoding beta-III-spectrin. To investigate the molecular basis of SCA5, we established a series of transgenic Drosophila models that express ..
  56. ncbi Clinical significance of alphaII-spectrin breakdown products in cerebrospinal fluid after severe traumatic brain injury
    Jose A Pineda
    Center for Traumatic Brain Injury Studies, E F and W L McKnight Brain Institute of the University of Florida, Gainesville, Florida, USA
    J Neurotrauma 24:354-66. 2007
    Following traumatic brain injury (TBI), the cytoskeletal protein alpha-II-spectrin is proteolyzed by calpain and caspase-3 to signature breakdown products...
  57. ncbi Modulation of the neuronal glutamate transporter EAAT4 by two interacting proteins
    M Jackson
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, Maryland 21287, USA
    Nature 410:89-93. 2001
    ....
  58. ncbi Spectrins and ankyrinB constitute a specialized paranodal cytoskeleton
    Yasuhiro Ogawa
    Department of Neuroscience, University of Connecticut Health Center, Farmington, Connecticut 06030, USA
    J Neurosci 26:5230-9. 2006
    ..strategy led to the identification of three novel components of the paranodal cytoskeleton: ankyrinB, alphaII spectrin, and betaII spectrin...
  59. ncbi Tyrosine phosphorylation of ionotropic glutamate receptors by Fyn or Src differentially modulates their susceptibility to calpain and enhances their binding to spectrin and PSD-95
    Y Rong
    Neuroscience Program, University of Southern California, Los Angeles 90089 2520, USA
    J Neurochem 79:382-90. 2001
    ..In addition, phosphorylation of NR2A and NR2B C-terminal fusion proteins by Fyn or Src enhanced their binding to spectrin and PSD-95...
  60. ncbi Plasmodium falciparum erythrocyte membrane protein 1 is anchored to the actin-spectrin junction and knob-associated histidine-rich protein in the erythrocyte skeleton
    S S Oh
    Department of Medicine, Section of Hematology Oncology Research, St Elizabeth s Medical Center, Tufts University School of Medicine, 736 Cambridge Street, 02135, Boston, MA, USA
    Mol Biochem Parasitol 108:237-47. 2000
    ..We demonstrate in this study that the cytoplasmic domain of PfEMP1 (VAR(CD)) binds to host spectrin and actin and to full-length KAHRP in vitro...
  61. pmc Glycosylation of erythrocyte spectrin and its modification in visceral leishmaniasis
    Sajal Samanta
    Cancer and Cell Biology Division, Council of Scientific and Industrial Research Indian Institute of Chemical Biology, Kolkata, India
    PLoS ONE 6:e28169. 2011
    ..identification of two high-molecular weight 9-O-acetylated sialoglycoproteins as human erythrocytic α- and β-spectrin. Total spectrin purified from erythrocytes of VL patients (spectrin(VL)) was reactive with Achatinin-H...
  62. pmc AlphaII-spectrin is an in vitro target for caspase-2, and its cleavage is regulated by calmodulin binding
    Björn Rotter
    INSERM U409, Faculté de Médecine Bichat Association Claude Bernard, 16 rue Henri Huchard, BP416, 75870 Paris, France
    Biochem J 378:161-8. 2004
    The spectrin-actin scaffold underlying the lipid bilayer is considered to participate in cell-shape stabilization and in the organization of specialized membrane subdomains...
  63. ncbi Cardiac muscle cell cytoskeletal protein 4.1: analysis of transcripts and subcellular location--relevance to membrane integrity, microstructure, and possible role in heart failure
    Pamela M Taylor-Harris
    Randall Division of Cell and Molecular Biophysics, King s College London, Guy s Campus, London SE1 1UL, UK
    Mamm Genome 16:137-51. 2005
    The spectrin-based cytoskeleton assembly has emerged as a major player in heart functioning; however, cardiac protein 4.1, a key constituent, is uncharacterized. Protein 4...
  64. ncbi Effects of positive AMPA receptor modulators on calpain-mediated spectrin degradation in cultured hippocampal slices
    Hussam Jourdi
    Neuroscience Program, University of Southern California, Los Angeles, CA 90089 2520, USA
    Neurochem Int 46:31-40. 2005
    ..As LTP is associated with calpain activation and spectrin degradation, we determined the effects of ampakine treatment of cultured hippocampal slices on spectrin ..
  65. pmc A widely expressed betaIII spectrin associated with Golgi and cytoplasmic vesicles
    M C Stankewich
    Department of Pathology, Yale University, New Haven, CT 06520, USA
    Proc Natl Acad Sci U S A 95:14158-63. 1998
    b>Spectrin is an important structural component of the plasma membrane skeleton. Heretofore-unidentified isoforms of spectrin also associate with Golgi and other organelles...
  66. ncbi Myne-1, a spectrin repeat transmembrane protein of the myocyte inner nuclear membrane, interacts with lamin A/C
    John M K Mislow
    Department of Pathology, The University of Chicago, Chicago, IL 60637, USA
    J Cell Sci 115:61-70. 2002
    ..This protein, myne-1 (myocyte nuclear envelope), is predicted to have seven spectrin repeats, an interrupted LEM domain and a single transmembrane domain at its C-terminus...
  67. pmc Separating the effects of internal friction and transition state energy to explain the slow, frustrated folding of spectrin domains
    Beth G Wensley
    Department of Chemistry, University of Cambridge, Lensfield Road, Cambridge CB2 1EW, United Kingdom
    Proc Natl Acad Sci U S A 109:17795-9. 2012
    The elongated three-helix bundle domains spectrin R16 and R17 fold some two to three orders of magnitude more slowly than their homologue R15...
  68. pmc Protein 4.2 binds to the carboxyl-terminal EF-hands of erythroid alpha-spectrin in a calcium- and calmodulin-dependent manner
    Catherine Korsgren
    Division of Hematology Oncology, Children s Hospital Boston and Dana Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 285:4757-70. 2010
    b>Spectrin and protein 4.1 cross-link F-actin protofilaments into a network called the membrane skeleton. Actin and 4.1 bind to one end of beta-spectrin...
  69. pmc Drosophilia spectrin. I. Characterization of the purified protein
    R Dubreuil
    Department of Cellular and Developmental Biology, Harvard University, Cambridge, Massachusetts 02138
    J Cell Biol 105:2095-102. 1987
    We purified a protein from Drosophila S3 tissue culture cells that has many of the diagnostic features of spectrin from vertebrate organisms: (a) The protein consists of two equimolar subunits (Mr = 234 and 226 kD) that can be reversibly ..
  70. pmc Slow, reversible, coupled folding and binding of the spectrin tetramerization domain
    S L Shammas
    Department of Chemistry, University of Cambridge, Cambridge, United Kingdom
    Biophys J 103:2203-14. 2012
    ..One such system is the association between α- and β-spectrin, where two partially structured, incomplete domains associate to form a fully structured, three-helix bundle, the ..
  71. ncbi High-resolution crystal structure of spectrin SH3 domain fused with a proline-rich peptide
    Liubov V Gushchina
    Institute of Protein Research, Russian Academy of Sciences, Pushchino, Moscow Region, Russia
    J Biomol Struct Dyn 29:485-95. 2011
    ..named WT-CIIA, was designed by connecting the proline-rich decapeptide PPPVPPYSAG to the C-terminus of the alpha-spectrin SH3 domain through a natural twelve-residue linker to obtain a single-chain model that would imitate ..
  72. pmc Serum concentrations of ubiquitin C-terminal hydrolase-L1 and αII-spectrin breakdown product 145 kDa correlate with outcome after pediatric TBI
    Rachel P Berger
    Children s Hospital of Pittsburgh of University of Pittsburgh Medical Center UPMC, Safar Center for Resuscitation Research, Pittsburgh, Pennsylvania 15224, USA
    J Neurotrauma 29:162-7. 2012
    ..to measure serum concentrations of two novel serum biomarkers, ubiquitin C-terminal hydrolase (UCH-L1) and αII-spectrin breakdown product 145 kDa (SBDP145), in children with TBI and healthy controls and to assess the ability of these ..
  73. pmc Apparent cooperativity in the folding of multidomain proteins depends on the relative rates of folding of the constituent domains
    Sarah Batey
    Department of Chemistry, University of Cambridge, Medical Research Council Centre for Protein Engineering, Lensfield Road, Cambridge CB2 1EW, UK
    Proc Natl Acad Sci U S A 103:18113-8. 2006
    ..We present the folding of pairs of three-helix bundle spectrin domains as a paradigm to indicate how complex such an analysis can be...
  74. pmc Role of insulin-dependent cortical fodrin/spectrin remodeling in glucose transporter 4 translocation in rat adipocytes
    Libin Liu
    Department of Biochemistry, Boston University Medical School, Boston, MA 02118, USA
    Mol Biol Cell 17:4249-56. 2006
    Fodrin or nonerythroid spectrin is an abundant component of the cortical cytoskeletal network in rat adipocytes...
  75. ncbi Specific role of the truncated betaIV-spectrin Sigma6 in sodium channel clustering at axon initial segments and nodes of ranvier
    Yoko Uemoto
    Department of Biological Sciences, Tokyo Institute of Technology, Yokohama 226 8501, Japan
    J Biol Chem 282:6548-55. 2007
    At axon initial segments and nodes of Ranvier in neurons, the spectrin membrane skeleton plays roles in physically stabilizing the plasma membrane integrity and in clustering voltage-gated sodium channels for proper conduction of the ..
  76. pmc The ring-infected erythrocyte surface antigen (RESA) of Plasmodium falciparum stabilizes spectrin tetramers and suppresses further invasion
    Xinhong Pei
    Red Cell Physiology Laboratory, New York Blood Center, 310 E 67th Street, New York, NY 10021, USA
    Blood 110:1036-42. 2007
    ..The protein migrates to the host cell membrane, where it binds to spectrin, but neither the nature of the interaction nor its functional consequences have previously been defined...
  77. pmc Activation of endosomal dynein motors by stepwise assembly of Rab7-RILP-p150Glued, ORP1L, and the receptor betalll spectrin
    Marie Johansson
    Department of Molecular Medicine, National Public Health Institute, Biomedicum, FI 00251 Helsinki, Finland
    J Cell Biol 176:459-71. 2007
    ..ORP1L, as well as betaIII spectrin, which is the general receptor for dynactin on vesicles, are essential for dynein motor activity...
  78. pmc The spectrin-based membrane skeleton stabilizes mouse megakaryocyte membrane systems and is essential for proplatelet and platelet formation
    Sunita Patel-Hett
    Translational Medicine Division, Department of Medicine, Brigham and Women s Hospital, Boston, MA, USA
    Blood 118:1641-52. 2011
    ..Although the functions of microtubules and actin during platelet biogenesis have been defined, the role of the spectrin cytoskeleton is unknown...
  79. ncbi Structure of the calmodulin alphaII-spectrin complex provides insight into the regulation of cell plasticity
    Miljan Simonovic
    Howard Hughes Medical Institute, Yale University, New Haven, Connecticut 06520, USA
    J Biol Chem 281:34333-40. 2006
    AlphaII-spectrin is a major cortical cytoskeletal protein contributing to membrane organization and integrity...
  80. ncbi Beta heavy-spectrin has a restricted tissue and subcellular distribution during Drosophila embryogenesis
    G H Thomas
    Department of Cellular and Developmental Biology, Harvard University, Cambridge, MA 02138
    Development 120:2039-50. 1994
    ..beta Heavy-spectrin is a unique beta-spectrin from Drosophila melanogaster that is closer in size (M(r) = 430 x 10(3)) to dystrophin ..
  81. ncbi Ankyrin-G and beta2-spectrin collaborate in biogenesis of lateral membrane of human bronchial epithelial cells
    Krishnakumar Kizhatil
    Howard Hughes Medical Institute and Departments of Cell Biology, Biochemistry, and Neurobiology, Duke University Medical Center, Durham, North Carolina 27710, USA
    J Biol Chem 282:2029-37. 2007
    ..In this study, we systematically generated alanine mutants of clusters of charged residues in the spectrin-binding domains of both ankyrin-B and -G...
  82. ncbi Defining of the minimal domain of protein 4.1 involved in spectrin-actin binding
    P O Schischmanoff
    Life Sciences Division, Lawrence Berkeley Laboratory, University of California, Berkeley 94720, USA
    J Biol Chem 270:21243-50. 1995
    The spectrin-actin-binding domain of protein 4.1 is encoded by a 21-amino acid alternative exon and a 59-amino acid constitutive exon...
  83. ncbi AlphaII-spectrin is critical for cell adhesion and cell cycle
    Sylvain Metral
    INSERM, U665, Paris, F 75015, France
    J Biol Chem 284:2409-18. 2009
    ..Using small interfering RNA strategy in the WM-266 cell line derived from human melanoma, we found that alphaII-spectrin deficiency is associated with a defect in cell proliferation, which is related to a cell cycle arrest at the G1 ..
  84. pmc Clustering of the neural cell adhesion molecule (NCAM) at the neuronal cell surface induces caspase-8- and -3-dependent changes of the spectrin meshwork required for NCAM-mediated neurite outgrowth
    Doreen Westphal
    Zentrum für Molekulare Neurobiologie, Universitatsklinikum Hamburg Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany
    J Biol Chem 285:42046-57. 2010
    ..cell surface leads to activation of caspase-8 and -3 followed by the cleavage of the sub-membranous brain spectrin meshwork, but not of the actin or tubulin cytoskeleton...
  85. ncbi A mutant alphaII-spectrin designed to resist calpain and caspase cleavage questions the functional importance of this process in vivo
    Fleur Meary
    INSERM, U665, Paris, France
    J Biol Chem 282:14226-37. 2007
    ..The ubiquitous alphaII-spectrin chain bears in its central region a unique domain that is sensitive to several proteases such as calpains or ..
  86. pmc Protein folding: adding a nucleus to guide helix docking reduces landscape roughness
    Beth G Wensley
    Department of Chemistry, University of Cambridge, Cambridge CB2 1EW, UK
    J Mol Biol 423:273-83. 2012
    The elongated three-helix-bundle spectrin domains R16 and R17 fold and unfold unusually slowly over a rough energy landscape, in contrast to the homologue R15, which folds fast over a much smoother, more typical landscape...
  87. ncbi Human alpha spectrin II and the FANCA, FANCC, and FANCG proteins bind to DNA containing psoralen interstrand cross-links
    L W McMahon
    Department of Pathology and Laboratory Medicine and Graduate School of Biomedical Sciences, UMDNJ New Jersey Medical School, Newark, New Jersey 07103, USA
    Biochemistry 40:7025-34. 2001
    ..We have recently identified the structural protein nonerythroid alpha spectrin (alphaSpIISigma) as a component of a nuclear protein complex in normal human cells which is involved in the ..
  88. ncbi Human alpha spectrin II and the Fanconi anemia proteins FANCA and FANCC interact to form a nuclear complex
    L W McMahon
    Department of Pathology, University of Medicine and Dentistry New Jersey Medical School, Newark, New Jersey 07103, USA
    J Biol Chem 274:32904-8. 1999
    ..We now show that p230 is a well known structural protein, human alpha spectrin II (alphaSpIISigma*), and that levels of alphaSpIISigma* are not only significantly reduced in FA-A cells but ..
  89. pmc Cloning and chromosomal localization of the human cytoskeletal alpha-actinin gene reveals linkage to the beta-spectrin gene
    H Youssoufian
    Whitehead Institute for Biomedical Research, Cambridge, MA
    Am J Hum Genet 47:62-72. 1990
    ..isoform of alpha-actinin (alpha A), a ubiquitous actin-binding protein that shares structural homology with spectrin and dystrophin...
  90. pmc Knockdown of mu-calpain in Fanconi anemia, FA-A, cells by siRNA restores alphaII spectrin levels and corrects chromosomal instability and defective DNA interstrand cross-link repair
    Pan Zhang
    Department of Pathology and Laboratory Medicine, New Jersey Medical School and Graduate School of Biomedical Sciences, University of Medicine and Dentistry of New Jersey, Newark, New Jersey 07103, USA
    Biochemistry 49:5570-81. 2010
    We have previously shown that there is a deficiency in the structural protein, nonerythroid alpha spectrin (alphaIISp), in cells from patients with Fanconi anemia (FA)...
  91. ncbi Reduced excitability in the dentate gyrus network of betaIV-spectrin mutant mice in vivo
    Raphael Winkels
    Institute of Clinical Neuroanatomy, Goethe University, Theodor Stern Kai 7, Frankfurt am Main, Germany
    Hippocampus 19:677-86. 2009
    The submembrane cytoskeletal meshwork of the axon contains the scaffolding protein betaIV-spectrin. It provides mechanical support for the axon and anchors membrane proteins...
  92. pmc Voltage-gated ion channels in the axon initial segment of human cortical pyramidal cells and their relationship with chandelier cells
    Maria Carmen Inda
    Departamento de Biologia Celular, Universidad Complutense de Madrid, Jose Antonio Novais 2, 28040 Madrid, Spain
    Proc Natl Acad Sci U S A 103:2920-5. 2006
    ..is a high density of Na+ channels distributed along the length of the AIS together with the associated proteins spectrin betaIV and ankyrin G. In contrast, Kv1...
  93. ncbi Selective release of calpain produced alphalI-spectrin (alpha-fodrin) breakdown products by acute neuronal cell death
    Satavisha Dutta
    Department of Neuroscience Therapeutics, Pfizer Global Research and Development, Ann Arbor, MI 48105, USA
    Biol Chem 383:785-91. 2002
    Activation of calpain results in the breakdown of alpha II spectrin (alpha-fodrin), a neuronal cytoskeleton protein, which has previously been detected in various in vitro and in vivo neuronal injury models...
  94. ncbi Spectrin mutations in spinocerebellar ataxia (SCA)
    Peter Bauer
    Department of Medical Genetics, University of Tubingen, Germany
    Bioessays 28:785-7. 2006
    ..The findings suggest that the mechanical properties of neurons and their dynamics may be as important as altered Ca(2+) homeostasis, transcriptional dysregulation, and impaired protein degradation in neurodegeneration conditions...
  95. ncbi The spectrin-ankyrin skeleton controls CD45 surface display and interleukin-2 production
    Deepti Pradhan
    Department of Pathology, Yale University, 310 Cedar Street, New Haven, CT 06510, USA
    Immunity 17:303-15. 2002
    With T cell receptor stimulation, intracellular pools of CD45 and spectrin move to the surface. These processes are coupled. In both peripheral lymphocytes and Jurkat T cells, betaI spectrin and ankyrin associate with CD45...
  96. ncbi Calpain modulates capacitation and acrosome reaction through cleavage of the spectrin cytoskeleton
    Yadira Bastián
    Deparment of Biology, McGill University, Montreal, Quebec, Canada
    Reproduction 140:673-84. 2010
    ..is relocated and translocated from cytoplasm to plasma membrane (PM) during capacitation, where it could cleave spectrin, one of the proteins of the PM-associated cytoskeleton, and facilitates AR...
  97. ncbi Structural insights into the stability and flexibility of unusual erythroid spectrin repeats
    Hideki Kusunoki
    Department of Biochemistry, Molecular Biology, and Cell Biology, Northwestern University, 2205 Tech Drive, Evanston, IL 60208 USA
    Structure 12:645-56. 2004
    Erythroid spectrin, a major component of the cytoskeletal network of the red cell which contributes to both the stability and the elasticity of the red cell membrane, is composed of two subunits, alpha and beta, each formed by 16-20 ..
  98. ncbi Binding of alphaII spectrin to 14-3-3beta is involved in NCAM-dependent neurite outgrowth
    Elisa M Ramser
    Zentrum fur Molekulare Neurobiologie Hamburg, Universitatsklinikum Hamburg Eppendorf, Martinistrasse 52, D 20246 Hamburg, Germany
    Mol Cell Neurosci 45:66-74. 2010
    ..chromatography followed by mass spectrometry analysis, we show here that the cytoskeletal protein alphaII spectrin is a novel ligand of 14-3-3beta...
  99. ncbi Brain spectrin: of mice and men
    S R Goodman
    Department of Structural and Cellular Biology, University of South Alabama College of Medicine, Mobile 36688, USA
    Brain Res Bull 36:593-606. 1995
    ..We discuss the known protein interactions of brain spectrin isoforms, and then describe results that suggest an important role for spectrin (alpha SpII sigma 1/beta SpII ..
  100. pmc Alpha spectrin is essential for morphogenesis and body wall muscle formation in Caenorhabditis elegans
    Kenneth R Norman
    Department of Zoology, University of British Columbia, Vancouver, BC, Canada V6T 1Z4
    J Cell Biol 157:665-77. 2002
    A common feature of multicellular animals is the ubiquitous presence of the spectrin cytoskeleton. Although discovered over 30 yr ago, the function of spectrin in non-erythrocytes has remained elusive...
  101. ncbi Crystal structure of a Src-homology 3 (SH3) domain
    A Musacchio
    European Molecular Biology Laboratory, Heidelberg, Germany
    Nature 359:851-5. 1992
    ..Here we report the three-dimensional structure at 1.8 A resolution of the SH3 domain of the cytoskeletal protein spectrin expressed in Escherichia coli. The domain is a compact beta-barrel made of five antiparallel beta-strands...

Research Grants63

  1. Mapping for Specialized Domains for FCeRI Signaling &Internalization
    Bridget S Wilson; Fiscal Year: 2013
    ..We have documented the expression of 2 a spectrin and 3 [unreadable] spectrin isoforms in RBL-2H3 cells...
  2. Right ventricular contractile dysfunction after pressure overload
    CLIFFORD RUSSELL GREYSON; Fiscal Year: 2013
    ..model, contractile dysfunction has been attributed to disruption of structural proteins such as desmin, spectrin, 1-actinin or titin;modification of regulatory proteins such as troponin-I, troponin-T;or initiation of apoptosis...
  3. Glutamate receptor recruitment to new synapses in vivo
    PHILIP ERIC WASHBOURNE; Fiscal Year: 2013
    ..We also propose that binding of DAL-1 to SynCAM1 or 2 in dendrites results in formation of an actin/spectrin subsynaptic scaffold...
  4. Molecular Biology of Human Erythrocyte Alpha Spectrin
    Patrick G Gallagher; Fiscal Year: 2013
    ..proposal are to elucidate the molecular mechanisms involved in normal and abnormal expression of the protein 1-spectrin, a critical component of the erythrocyte membrane skeleton...
  5. MINORITY PREDOCTORAL FELLOWSHIP PROGRAM
    Quentin J Baca; Fiscal Year: 2010
    ..We will also investigate the role of specific molecular partners (e.g., spectrin, ankyrin) in CD45 mobility regulation...
  6. BRAINSTEM MATURATION IN THE SUDDEN INFANT DEATH SYNDROME
    HANNAH CHASE KINNEY; Fiscal Year: 2013
    ..transduction, including in regulation of TPH2;and 2) certain synaptic proteins, including 3-synuclein, actin, and spectrin. Our over-riding hypothesis is that an important subset of SIDS is due to alterations in key proteins related to ..
  7. Examination of the Final Stages of Platelet Production
    JONATHAN NOAH THON; Fiscal Year: 2013
    ..work and focus on protein translation/degradation pathways that initiate proPLT production, and the role of the spectrin- based membrane skeleton on PLT formation...
  8. Dissecting the role of one neuronal RhoGEF amongst many: the Kalirin-7 null mouse
    Richard E Mains; Fiscal Year: 2012
    ..by expressing exogenous Kalirin-7, allowing detailed analysis of the role of Kalirin-7 and the isolated Sec14p, spectrin-like, DH and PH domains...
  9. Role of spectrin/ankyrin-G complex in myocyte signaling and cardiac excitability
    Peter J Mohler; Fiscal Year: 2013
    ..bIV spectrin, an actin- and ankyrin-G-associated molecule, associates with CaMKIId both in vitro and in vivo...
  10. Role of Beta Spectrin and Smad in Alcohol Induced Liver and GI Cell Proliferation
    Lopa Mishra; Fiscal Year: 2013
    ..c) Expand to fine mapping. Significantly, studies proposed under this application should provide the scientific community with a new understanding of the instrumental regulators in alcohol induced liver injury and neoplasia. ..
  11. Membrane skeleton regulation of cell shape and interactions in lens development
    Velia M Fowler; Fiscal Year: 2013
    ..A cross-linked network of spectrin and actin filaments (F-actin), attaches to NrCAM and N-cadherin, creating micron-scale membrane subdomains in ..
  12. In vivo mechanisms of methamphetamine-induced neuronal necrosis
    Denson G Fujikawa; Fiscal Year: 2013
    ..nuclear cyt c, cath-B and cath-D;(4) fluorometric calpain activity assays and western blots of [unreadable]II-spectrin, a calpain substrate, to document calpain I activation;(5) parentera administration of diazepam and phenobarbital ..
  13. Cytoskeletal Pliability within Cells under Stress: RBCs and Marrow-derived SCs
    Dennis E Discher; Fiscal Year: 2012
    ..stresses affect normal and diseased RBC cytoskeleton, and show with intact membranes that specific domains in spectrin unfold under fluid shear...
  14. Red Cell Deformability InVitro and Survival InVivo
    MOHANDAS NARLA; Fiscal Year: 2013
    ..In particular, we will explore the dynamic regulation of linkage between spectrin dimers and between junctional complex proteins, spectrin, 4.1R, actin and adducin...
  15. DYNAMIC INTERATION AMONG PROTEINS IN HAIR CELLS
    Jing Zheng; Fiscal Year: 2013
    ..of interactions between Marshalin and its potential associated proteins including CDH23, actin, myosin 7a and spectrin. The investigation will provide much-needed information regarding Marshalin's roles in cross-linking actin ..
  16. Relative Roles of HSP70 and HSP25 in the Modulation of Renal Injury
    Rajasree Sreedharan; Fiscal Year: 2010
    ..with a panel of proteins involved in maintenance of cellular integrity and polarity (NaK ATPase, ankyrin, spectrin and actin) and c) the molecular processes by which HSP interactions with these integrity related proteins result ..
  17. Genes in Human Brain Ischemia
    W Andrew Kofke; Fiscal Year: 2011
    ..H, hypophospho-neurofilament H, dephospho- neurofilament H, and a calpain-derived N-terminal fragment of alpha-spectrin). H2...
  18. Cytoskeletal Regulation of Erythrocyte Glucose Transporter-1
    Athar H Chishti; Fiscal Year: 2012
    SUMMARY Erythrocyte spectrin-actin junctions play a critical role in the maintenance of cell shape and membrane properties. Their organization and membrane linkage is therefore of fundamental importance to erythrocyte physiology...
  19. Neuronal Somatic Response to Traumatic Brain Injury
    John T Povlishock; Fiscal Year: 2010
    ..Concomitant immunocytochemical detection of calpain-mediated spectrin proteolysis, 5-calpain activation, lysosomal associated membrane protein release, and/or cathepsin B activation ..
  20. Dystrophin Function in Aging Heart
    JOSEPH MARK METZGER; Fiscal Year: 2013
    ..Hypothesis: truncated dystrophin with optimized hinge and spectrin-like repeat domains will confer increased cardiac performance relative to first generation micro-dystrophin in ..
  21. Fiber Cell Cytoskeletal Scaffolds-Role in Lens Architecture and Function
    P Vasantha Rao; Fiscal Year: 2011
    ..Interestingly, co-immunoprecipitates of PRX from lens fibers contain ankyrin B, spectrin, filensin, desmoyokin, NrCAM and aquaporin-0, suggesting a role for PRX in fiber cell membrane network ..
  22. The role of nucleo-cytoskeletal link proteins in skeletal muscle
    Ju Chen; Fiscal Year: 2013
    ..Nesprins belong to a newly discovered family of mammalian spectrin-repeat proteins. Mice lacking Lamin A/C exhibit features of EDMD...
  23. Capping Actin Growth in Erythroid and Nonerythroid Cells
    Velia M Fowler; Fiscal Year: 2013
    ..applicant): The long-range goal of this research is to determine how actin filament dynamics is regulated in the spectrin-actin lattice of the membrane skeleton to create the red blood cell (RBC) with its specialized membrane, unique ..
  24. Structural studies of a spectrin/ankyrin complex
    ALFONSO MONDRAGON; Fiscal Year: 2013
    ..b>Spectrin is a key element of this network...
  25. CaMKII-dependent regulation of cardiac excitability
    THOMAS JEFFREY HUND; Fiscal Year: 2013
    ..We recently demonstrated that the actin-associated polypeptide betaIV-spectrin serves as a novel CaMKII-anchoring protein (CaMKAP), which targets CaMKII to the intercalated disc for regulation ..
  26. Membrane Proteins of Normal and Abnormal Red Cells
    David W Speicher; Fiscal Year: 2013
    ..protein network (membrane skeleton) on the cytoplasmic face of the membrane, which is comprised primarily of spectrin tetramers that crosslink short actin filaments...
  27. A comprehensive RNAi based screen for genes effecting neural degeneration in C. e
    Michael Bastiani; Fiscal Year: 2009
    ..elegans. We discovered that embryonic neurons lacking -spectrin develop normally (normal growth cone motility, pathfinding, and target recognition), but after hatching undergo a ..
  28. Therapeutic Strategies for Spinal Cord Injury
    Radhika Vaishnav; Fiscal Year: 2009
    ..Calpain-mediated cytoskeletal breakdown will be assessed using an immunochemical spectrin breakdown assay as an indirect measure of calpain activity...
  29. Cytoskeletal Mechanisms of Platelet Formation
    Joseph E Italiano; Fiscal Year: 2013
    ..a new intermediate stage in platelet production, the preplatelet, and have established that microtubules and the spectrin-based membrane skeleton are involved in platelet production...
  30. Spectrin-based signaling complex regulates cardiac excitability
    THOMAS JEFFREY HUND; Fiscal Year: 2012
    ..Specifically, we have identified beta lV-spectrin as a novel intercalated disc CaMKII anchoring protein that complexes CaMKII with Nav1...
  31. Biochemical Markers of Traumatic Brain Injury
    STEVEN ANDREW ROBICSEK; Fiscal Year: 2010
    ..Aim 1 will assess the diagnostic and prognostic utility of candidate biomarkers (breakdown products to alpha-spectrin-SBDPs;UCH-L1;MAP2a/b;myelin basic protein-MBP;S100B) by examining relationships between levels of specific ..
  32. Nesprin 1 and 2 in cardiac structure, function, and disease
    Ju Chen; Fiscal Year: 2013
    ..Nesprins belong to a newly discovered family of spectrin-repeat proteins, composed of four members...
  33. Molecular and Cellular Mechanisms of Synaptic Growth
    Graeme W Davis; Fiscal Year: 2012
    ..the mutations in the presynaptic Dynein/Dynactin protein complex cause NMJ degeneration, as do mutations in the Spectrin/Ankyrin skeleton...
  34. DETERMINANTS AND CONSEQUENCES OF MDMA NEUROTOXICITY
    Gary Gudelsky; Fiscal Year: 2010
    ..and 4) assess the extent and mechanism by which MDMA promotes the cleavage of the cytoskeletal proteins tau and spectrin. The linkage of the processes of oxidative damage and mitochondrial impairment to the long-term effects of MDMA ..
  35. Functional Studies of Erythrocyte Dematin
    Athar H Chishti; Fiscal Year: 2010
    It is well recognized that the "spectrin-actin junctions" are functionally essential for the maintenance of erythrocyte membrane stability and mechanical properties...
  36. Molecular Basis of Sensory Transduction in C. elegans
    Miriam B Goodman; Fiscal Year: 2013
    ..1) alter biosynthesis of polyunsaturated fatty acids and cell membrane function, 2) disrupt the microtubule and spectrin cytoskeleton and 3) disrupt selected domains in the MEC-4 proteins crucial to the formation of the sodium-..
  37. Store-operated calcium entry: lung endothelial permeability
    Troy Stevens; Fiscal Year: 2013
    ..The TRPC4 subunits within this channel bind to protein 4.1, and protein 4.1 tethers the channel to the spectrin membrane skeleton...
  38. TLR4 and TGF-beta interactions in HCC induced by HCV and alcohol
    Hidekazu Tsukamoto; Fiscal Year: 2010
    ..Defective TGF-2 signaling in 22 spectrin heterozygote (22sp+/-) mice, leads to aberrant IL-6 and Nanog induction and spontaneous HCC development...
  39. Novel calpain inhibitor therapy for traumatic brain injury
    Yasuhiro Maeda; Fiscal Year: 2013
    ..dye fluorescence), glial and immune/inflammatory responses (cell specific markers) as well as quantification of spectrin breakdown products by Western Immunoblot assay...
  40. Neuroglial Interactions at the Node of Ranvier
    Matthew N Rasband; Fiscal Year: 2013
    ..In particular, nodes of Ranvier themselves are enriched with ankyrinG (ankG) and ?IV spectrin;ankG is thought to bind directly to the Na+ and K+ channels, and then link to the actin cytoskeleton through ?IV ..
  41. Role of Necl-4 Complex PNS Myelination
    XIAOSONG MENG; Fiscal Year: 2012
    ..1G and its association with spectrin and the actin cytoskeleton, thereby promoting long-term viability of the myelin sheath...
  42. Mechanisms that Regulate Intracellular Transport
    Thomas S Hays; Fiscal Year: 2013
    ..We recently showed that ss-spectrin mutations in humans that cause spinocerebellar ataxia type 5 (SCA5) impair motor-based transport of synaptic ..
  43. INHIBITION OF NONERYTHROID SPECTRIN BY ANTISENSE RNA
    Randall Moon; Fiscal Year: 1993
    ..The major protein in this membrane skeleton is spectrin, which is a very large, flexible, rod-like dimeric protein...
  44. Assembly of Spectrin Isoforms
    LESLIE FUNG; Fiscal Year: 2009
    Our broad, long-term objectives are to understand conformations of critical regions in various spectrin isoforms and to correlate specific structural features in these isoforms with functions unique to individual isoforms...
  45. ARCHITECTURE OF ERYTHROCYTE SKELETON--BILAYER INTERFACE
    STEPHEN SHOHET; Fiscal Year: 2000
    DESCRIPTION: (Adapted from Applicant's Abstract) Dr. Shohet hypothesizes that the distance between the spectrin-based membrane skeleton and the bilayer increases in certain types of hereditary spherocytosis due to defects or deficiencies ..
  46. Molecular Genetics of Cytoplasmic Dyein
    RICHARD BERT VALLEE; Fiscal Year: 2010
    ..for ZW10 membrane binding;focusing on the Golgi apparatus, we will determine the role of ZW10 relative to Rab6, spectrin, and other factors implicated in dynein cargo binding, with the goal of a complete elucidation of the Golgi cargo ..
  47. GENETIC ANALYSIS OF CHARCOT-MARIE-TOOTH DISEASE
    PHILLIP CHANCE; Fiscal Year: 1992
    ..in multiple large HMSNIb families with known gene markers from chromosome 1 including: alpha actin, alpha spectrin, amylase, amyloid P, antithrombin III, apolipoprotein AII, H4 histone, N-ras, C-reactive protein and p308...
  48. PHOSPHOINOSITIDE REGULATION OF ERYTHROCYTE CYTOSKELETON
    Richard Andersen; Fiscal Year: 1991
    ..1, and spectrin-4.1-actin, will then associate with the cytoplasmic segment of glycophorin...
  49. BIOGENESIS OF THE RED CELL MEMBRANE AND CYTOSKELETON
    Hagop Youssoufian; Fiscal Year: 1993
    ..The erythroid proteins spectrin and band 3, for example, remain with the mature reticulocyte, while most surface proteins segregate with the ..
  50. MOLECULAR INTERACTIONS OF BRAIN SPECTRIN
    Leszek Kotula; Fiscal Year: 1999
    ..function and regulatory processes of the neuronal cell membrane skeleton and its major component - brain spectrin. Neuronal compartmentalization of brain spectrin isoforms into axons and presynaptic terminals (nonerythroid ..
  51. THE ROLE OF CONTRACTILE PROTEINS IN ERYTHROCYTE AGING
    DIANE LIN; Fiscal Year: 1980
    The Spectrin and actin network on the cytoplasmic side of the red cell membrane is believed to be an important determinant of cell deformability and osmotic fragility...
  52. RED CELL MEMBRANE SKELETON; ULTRASTRUCTURE & FUNCTION
    Betty Shen; Fiscal Year: 1992
    ..specimens that the skeleton is organized into short actin protofilaments interconnected by multiple strands of spectrin tetramer...
  53. PATHOPHYSIOLOGY OF RED CELL MEMBRANE DISEASE
    SAMUEL LUX; Fiscal Year: 2003
    DESCRIPTION: ( Investigator's abstract) In hereditary spherocytosis (HS), defects of spectrin ankyrin, band 3 and protein 4.2 lead to spheroidal, osmotically fragile cells that are selectively trapped in the spleen...
  54. Regulation of GPIIb-IIIa by the platelet cytoskeleton
    Joan Fox; Fiscal Year: 2003
    ..evidence that a subpopulation of a11bB3 in platelets is associated with membrane skeleton complexes containing spectrin, talin, pp60c-src, and calpain...
  55. Hemolytic Anemia: Models
    LUANNE PETERS; Fiscal Year: 2006
    ..Of the four domains--membrane binding, spectrin binding, death, and regulatory--the COOH-terminal regulatory region is the most divergent, provides the highest ..
  56. INHIBITION OF NONERYTHROID SPECTRIN BY ANTISENSE RNA
    Randall Moon; Fiscal Year: 1991
    ..The major protein in this membrane skeleton is spectrin, which is a very large, flexible, rod-like dimeric protein...
  57. Novel Biomarkers for Stroke Injury
    MONIKA OLI; Fiscal Year: 2005
    ..We have developed a specific and sensitive biomarker for acute ischemic stroke. all-Spectrin is a major substrate for calpain and caspase-3 proteolysis, which each produce a unique 150 kDa spectrin ..
  58. Topographic Spectrin Assembly in the Renal Tubule Cell
    Jon Morrow; Fiscal Year: 2007
    ..The spectrin-based membrane skeleton organizes mosaics of membrane proteins and lipids, and tethers them to cytoskeletal ..
  59. SPECTRIN-LIKE PROTEIN IN DEVELOPING BRAIN
    IAN ZAGON; Fiscal Year: 1991
    This project is designed to explore spectrin and associated cytoskeletal proteins in regard to the structure and function of the developing nervous system...
  60. BRAIN SPECTRIN AND SYNAPTIC TRANSMISSION
    Steven Goodman; Fiscal Year: 2000
    This is a new application to study the role of brain spectrin in synaptic transmission. We will determine whether spectrin plays a direct role in depolarization-induced release of neurotransmitter from the presynaptic terminal...
  61. MEMBRANE LIPID MATRIX, CYTOSKELETON AND IMMUNE FUNCTION
    Elizabeth Repasky; Fiscal Year: 1990
    ..from this laboratory concerning unexpectedly large differences in the amount of the cytoskeletal protein spectrin associated with the inner surface of the plasma membrane among lymphocyte subsets in vivo and in vitro...
  62. MOLECULAR GENETICS OF HUMAN RED BLOOD CELL SPECTRIN
    JOHN WINKELMANN; Fiscal Year: 1992
    ..normal and pathologic expression of the genes for the major human red blood cell membrane skeleton protein spectrin. Alpha and beta spectrin mRNA levels, synthesis and degradation will be measured in normal red cell maturation...
  63. ISOLATION & CHARACTERIZATION OF LENS SPECTRIN & ANKYRINN
    Josef Prchal; Fiscal Year: 1992
    ..b>Spectrin and ankyrin, major protein components of the cytoskeletal architecture, have recently been identified in the ..