Experts and Doctors on spectrin in United States


Locale: United States
Topic: spectrin

Top Publications

  1. Deng Bryant Y, Singh I, Carrico K, Hall E. Neuroprotective effects of tempol, a catalytic scavenger of peroxynitrite-derived free radicals, in a mouse traumatic brain injury model. J Cereb Blood Flow Metab. 2008;28:1114-26 pubmed publisher
    ..However, the modest neuroprotective effect of tempol suggests that multitarget combination strategies may be needed to interfere with posttraumatic secondary injury to a degree worthy of clinical translation. ..
  2. Armstrong S, Latham C, Shivell C, Ganote C. Ischemic loss of sarcolemmal dystrophin and spectrin: correlation with myocardial injury. J Mol Cell Cardiol. 2001;33:1165-79 pubmed
  3. Karmakar S, Banik N, Ray S. Curcumin suppressed anti-apoptotic signals and activated cysteine proteases for apoptosis in human malignant glioblastoma U87MG cells. Neurochem Res. 2007;32:2103-13 pubmed
    ..Results show that CCM is an effective therapeutic agent for suppression of anti-apoptotic factors and activation of calpain and caspase proteolytic cascades for apoptosis in human malignant glioblastoma cells. ..
  4. Das A, Sribnick E, Wingrave J, Del Re A, Woodward J, Appel S, et al. Calpain activation in apoptosis of ventral spinal cord 4.1 (VSC4.1) motoneurons exposed to glutamate: calpain inhibition provides functional neuroprotection. J Neurosci Res. 2005;81:551-62 pubmed
    ..Inhibition of the activities of both NMDA and AMPA receptors protected VSC4.1 cells from glutamate toxicity and preserved whole-cell membrane potential. ..
  5. Sur P, Sribnick E, Wingrave J, Nowak M, Ray S, Banik N. Estrogen attenuates oxidative stress-induced apoptosis in C6 glial cells. Brain Res. 2003;971:178-88 pubmed
    ..Our results suggest that estrogen may have a protective role in minimizing glial cell apoptosis in neurological diseases such as demyelinating disease or central nervous system trauma. ..
  6. Xiong Y, Hall E. Pharmacological evidence for a role of peroxynitrite in the pathophysiology of spinal cord injury. Exp Neurol. 2009;216:105-14 pubmed publisher
  7. Schoch K, von Reyn C, Bian J, Telling G, Meaney D, Saatman K. Brain injury-induced proteolysis is reduced in a novel calpastatin-overexpressing transgenic mouse. J Neurochem. 2013;125:909-20 pubmed publisher
    ..Augmenting calpastatin levels may be an effective method for calpain inhibition in traumatic brain injury and neurodegenerative disorders. ..
  8. Lewis S, Velat G, Miralia L, Papa L, Aikman J, Wolper R, et al. Alpha-II spectrin breakdown products in aneurysmal subarachnoid hemorrhage: a novel biomarker of proteolytic injury. J Neurosurg. 2007;107:792-6 pubmed
    ..Differential expression of SBDPs suggests oncotic necrotic proteolysis may be predominant in acute brain injury after ASAH and cerebral arterial vasospasm. ..
  9. Shaw G. The pleckstrin homology domain: an intriguing multifunctional protein module. Bioessays. 1996;18:35-46 pubmed
    ..Numerous recent studies show that PH domains bind various proteins and inositolphosphates. Here I discuss PH domains in detail and conclude that they form a versatile family of membrane binding and protein localization modules. ..

More Information


  1. Schaecher K, Rocchini A, Dinkins J, Matzelle D, Banik N. Calpain expression and infiltration of activated T cells in experimental allergic encephalomyelitis over time: increased calpain activity begins with onset of disease. J Neuroimmunol. 2002;129:1-9 pubmed
    ..This study demonstrates that cell infiltration and increased calpain activity do not begin in the CNS until the onset of clinical signs. ..
  2. Fukushima Y, Byers M, Watkins P, Winkelmann J, Forget B, Shows T. Assignment of the gene for beta-spectrin (SPTB) to chromosome 14q23----q24.2 by in situ hybridization. Cytogenet Cell Genet. 1990;53:232-3 pubmed
    ..1988). Here we report the regional localization of this gene by in situ hybridization to 14q23----q24.2. ..
  3. Kassa R, Monterroso V, Wentzell J, Ramos A, Couchi E, Lecomte M, et al. Proximal giant neurofilamentous axonopathy in mice genetically engineered to resist calpain and caspase cleavage of ?-II spectrin. J Mol Neurosci. 2012;47:631-8 pubmed publisher
    ..Our conceptual framework offers opportunities to study the role of calpain-caspase cross talk, including that of the protease degradomics, in models of axonal degeneration. ..
  4. Lefferts J, Wang C, Sridharan D, Baralt M, Lambert M. The SH3 domain of alphaII spectrin is a target for the Fanconi anemia protein, FANCG. Biochemistry. 2009;48:254-63 pubmed publisher
    ..We propose that this binding of FANCG to alphaIISp may be important for the stability of alphaIISp in cells and the role alphaIISp plays in the DNA repair process. ..
  5. McPherson C, Eipper B, Mains R. Multiple novel isoforms of Trio are expressed in the developing rat brain. Gene. 2005;347:125-35 pubmed
    ..Trio/duet is expressed in adult cortex and cerebellum. In the rat brain, each of these Trio isoforms is expressed at a higher level than full length Trio. ..
  6. McMahon L, Sangerman J, Goodman S, Kumaresan K, Lambert M. Human alpha spectrin II and the FANCA, FANCC, and FANCG proteins bind to DNA containing psoralen interstrand cross-links. Biochemistry. 2001;40:7025-34 pubmed
  7. Zaccai M, Lipshitz H. Role of Adducin-like (hu-li tai shao) mRNA and protein localization in regulating cytoskeletal structure and function during Drosophila Oogenesis and early embryogenesis. Dev Genet. 1996;19:249-57 pubmed
  8. Derbala M, Guo A, Mohler P, Smith S. The role of ?II spectrin in cardiac health and disease. Life Sci. 2018;192:278-285 pubmed publisher
    ..This review focuses on our current knowledge of the role ?II spectrin plays in the cardiovascular system in health and disease and the potential future clinical implications. ..
  9. Knierim E, Gill E, Seifert F, Morales Gonzalez S, Unudurthi S, Hund T, et al. A recessive mutation in beta-IV-spectrin (SPTBN4) associates with congenital myopathy, neuropathy, and central deafness. Hum Genet. 2017;136:903-910 pubmed publisher
    ..More patients have to be described to confirm the triad of congenital myopathy, neuropathy and deafness as the defining symptom complex for ?IV-spectrin deficiency. ..
  10. King M, Phillips G, Bignone P, Hayes N, Pinder J, Baines A. A conserved sequence in calmodulin regulated spectrin-associated protein 1 links its interaction with spectrin and calmodulin to neurite outgrowth. J Neurochem. 2014;128:391-402 pubmed publisher
    ..We conclude that CC1 represents a functional region of CAMSAP1, which links spectrin-binding to neurite outgrowth. ..
  11. Taylor L, Kaminoh Y, Rodesch C, Flanigan K. Quantification of dystrophin immunofluorescence in dystrophinopathy muscle specimens. Neuropathol Appl Neurobiol. 2012;38:591-601 pubmed publisher
    ..This method offers a robust and reliable method of biomarker detection for trials of DMD therapies. ..
  12. Hong W, Doyle D. Cloning and analysis of cDNA clones for rat kidney alpha-spectrin. J Biol Chem. 1989;264:12758-64 pubmed
  13. Zhang P, Sridharan D, Lambert M. 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. Biochemistry. 2010;49:5570-81 pubmed publisher
    ..Thus, by reducing the level of breakdown of alphaIISp in FA cells, we may be able to reverse a number of the cellular deficiencies observed in this disorder. ..
  14. Gregorio C, Repasky E, Fowler V, Black J. Dynamic properties of ankyrin in T lymphocytes: colocalization with spectrin and protein kinase C beta. J Cell Biol. 1994;125:345-58 pubmed
    ..Our data support the conclusion that ankyrin is not solely involved in anchorage of proteins at the plasma membrane in lymphoid cells. ..
  15. Sihag R, Shea T, Wang F. Spectrin-actin interaction is required for neurite extension in NB 2a/dl neuroblastoma cells. J Neurosci Res. 1996;44:430-7 pubmed
    ..These results suggest that beta-spectrin-actin interaction may be required for neurite extension, which is critical for development of polarity in nerve cells. ..
  16. Zaccai M, Lipshitz H. Differential distributions of two adducin-like protein isoforms in the Drosophila ovary and early embryo. Zygote. 1996;4:159-66 pubmed
    ..ADD-87 co-localises with F-actin and spectrin in the cortex of the oocyte through stage 10 of oogenesis, consistent with a possible role in cytoskeletal assembly or function predicted by mammalian studies. ..
  17. Hedstrom K, Xu X, Ogawa Y, Frischknecht R, Seidenbecher C, Shrager P, et al. Neurofascin assembles a specialized extracellular matrix at the axon initial segment. J Cell Biol. 2007;178:875-86 pubmed
    ..Thus, NF-186 assembles and links the specialized brevican-containing AIS extracellular matrix to the intracellular cytoskeleton. ..
  18. Peters L, Jindel H, Gwynn B, Korsgren C, John K, Lux S, et al. Mild spherocytosis and altered red cell ion transport in protein 4. 2-null mice. J Clin Invest. 1999;103:1527-37 pubmed
    ..The increased passive Na+ permeability of 4.2(-/-) RBCs is also dependent on cell shrinkage. We conclude that protein 4.2 is important in the maintenance of normal surface area in RBCs and for normal RBC cation transport. ..
  19. Yang Y, Ogawa Y, Hedstrom K, Rasband M. betaIV spectrin is recruited to axon initial segments and nodes of Ranvier by ankyrinG. J Cell Biol. 2007;176:509-19 pubmed
    ..Finally, using adenovirus for transgene delivery into myelinated neurons, we demonstrate that betaIV spectrin recruitment to nodes of Ranvier also depends on binding to ankG. ..
  20. Aikman J, O Steen B, Silver X, Torres R, Boslaugh S, Blackband S, et al. Alpha-II-spectrin after controlled cortical impact in the immature rat brain. Dev Neurosci. 2006;28:457-65 pubmed
    ..Distinct proteolytic products resulting from the degradation of the cytoskeletal protein alpha-II-spectrin by calpain and caspase 3 were readily detectable in cortical brain parenchyma and cerebrospinal fluid after TBI in immature rats. ..
  21. Zarnescu D, Thomas G. Apical spectrin is essential for epithelial morphogenesis but not apicobasal polarity in Drosophila. J Cell Biol. 1999;146:1075-86 pubmed
  22. Masso Welch P, Black J, Erikson J, Repasky E. Polarized expression of immunoglobulin, spectrin, and protein kinase C beta II occurs in B cells from normal BALB/c, autoimmune lpr, and anti-ssDNA transgenic, tolerant mice. J Leukoc Biol. 1999;66:617-24 pubmed
    ..These data suggest that polarization of surface receptors, signaling molecules, anionic phospholipid domains, and cytoskeletal proteins may be an important part of the B cell immune response in vivo. ..
  23. McMahon L, Walsh C, Lambert M. Human alpha spectrin II and the Fanconi anemia proteins FANCA and FANCC interact to form a nuclear complex. J Biol Chem. 1999;274:32904-8 pubmed
    ..alphaSpIISigma* may thus act as a scaffold to align or enhance interactions between FA proteins and proteins involved in DNA repair. These results suggest that FA represents a disorder in which there is a deficiency in alphaSpIISigma*. ..
  24. Lefferts J, Lambert M. Fanconi anemia cell lines deficient in alphaII spectrin express normal levels of alphaII spectrin mRNA. Biochem Biophys Res Commun. 2003;307:510-5 pubmed
  25. Franco S, Perrin B, Huttenlocher A. Isoform specific function of calpain 2 in regulating membrane protrusion. Exp Cell Res. 2004;299:179-87 pubmed
    ..Together, our findings support a novel role for calpain 2 in limiting membrane protrusions and in regulating lamellipodial dynamics at the leading edge of migrating cells. ..
  26. Parkinson N, Olsson C, Hallows J, McKee Johnson J, Keogh B, Noben Trauth K, et al. Mutant beta-spectrin 4 causes auditory and motor neuropathies in quivering mice. Nat Genet. 2001;29:61-5 pubmed
  27. Sridharan D, Brown M, Lambert W, McMahon L, Lambert M. Nonerythroid alphaII spectrin is required for recruitment of FANCA and XPF to nuclear foci induced by DNA interstrand cross-links. J Cell Sci. 2003;116:823-35 pubmed
    ..These studies suggest that an important role for alphaSpIISigma* in the nucleus is to act as a scaffold, aiding in recruitment and alignment of repair proteins at sites of damage. ..
  28. Amor V, Feinberg K, Eshed Eisenbach Y, Vainshtein A, Frechter S, Grumet M, et al. Long-term maintenance of Na+ channels at nodes of Ranvier depends on glial contact mediated by gliomedin and NrCAM. J Neurosci. 2014;34:5089-98 pubmed publisher
  29. Kline C, Wright P, Koval O, Zmuda E, Johnson B, Anderson M, et al. ?IV-Spectrin and CaMKII facilitate Kir6.2 regulation in pancreatic beta cells. Proc Natl Acad Sci U S A. 2013;110:17576-81 pubmed publisher
  30. Subrahmanyam G, Bertics P, Anderson R. Phosphorylation of protein 4.1 on tyrosine-418 modulates its function in vitro. Proc Natl Acad Sci U S A. 1991;88:5222-6 pubmed
    ..1 occurs in vivo and may be functionally significant. The tyrosine phosphorylation site is in the center of a sequence motif that is expressed by a differentiation-specific splicing mechanism. ..
  31. Pike B, Flint J, Dave J, Lu X, Wang K, Tortella F, et al. Accumulation of calpain and caspase-3 proteolytic fragments of brain-derived alphaII-spectrin in cerebral spinal fluid after middle cerebral artery occlusion in rats. J Cereb Blood Flow Metab. 2004;24:98-106 pubmed
  32. Karmakar S, Banik N, Patel S, Ray S. Garlic compounds induced calpain and intrinsic caspase cascade for apoptosis in human malignant neuroblastoma SH-SY5Y cells. Apoptosis. 2007;12:671-84 pubmed
    ..Results strongly suggested that the garlic compounds DAS and DADS suppressed anti-apoptotic factors and activated calpain and intrinsic caspase cascade for apoptosis in SH-SY5Y cells. ..
  33. Mondello S, Robicsek S, Gabrielli A, Brophy G, Papa L, Tepas J, et al. ?II-spectrin breakdown products (SBDPs): diagnosis and outcome in severe traumatic brain injury patients. J Neurotrauma. 2010;27:1203-13 pubmed publisher
    ..The time course of SBDPs in nonsurvivors also differed from that of survivors. These results suggest that CSF SBDP levels can predict injury severity and mortality after severe TBI, and can be useful complements to clinical assessment. ..
  34. Yang Y, Lacas Gervais S, Morest D, Solimena M, Rasband M. BetaIV spectrins are essential for membrane stability and the molecular organization of nodes of Ranvier. J Neurosci. 2004;24:7230-40 pubmed
    ..These results show that quivering mutations disrupt betaIV spectrin retention and stability at nodes and that distinct protein domains regulate nodal structural integrity and molecular organization. ..
  35. Ogawa Y, Schafer D, Horresh I, Bar V, Hales K, Yang Y, et al. Spectrins and ankyrinB constitute a specialized paranodal cytoskeleton. J Neurosci. 2006;26:5230-9 pubmed
    ..Our results demonstrate that the paranodal junction contains specialized cytoskeletal components that may be important to stabilize axon-glia interactions and contribute to the membrane protein diffusion barrier found at paranodes. ..
  36. Sridharan D, McMahon L, Lambert M. alphaII-Spectrin interacts with five groups of functionally important proteins in the nucleus. Cell Biol Int. 2006;30:866-78 pubmed
    ..These studies indicate that alphaSpIISigma( *) may play a role in a number of diverse and important processes in the nucleus and that a deficiency in this protein, as occurs in FA, could affect a number of critical cellular pathways. ..
  37. Kamynina E, Kauppinen K, Duan F, Muakkassa N, Manor D. Regulation of proto-oncogenic dbl by chaperone-controlled, ubiquitin-mediated degradation. Mol Cell Biol. 2007;27:1809-22 pubmed
    ..Onco-Dbl, lacking the spectrin homology domain, cannot bind these regulators and therefore accumulates in cells at high levels, leading to persistent stimulation of its downstream signaling pathways. ..