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Mutation of a Highly Conserved Residue of beta I Spectrin Associated with Fatal and Near-fatal Neonatal Hemolytic ... I-spectrin gene and in exons 30 and 31 of the I-spectrin gene and exons 30, 31, and 32 of the Bennett(1985)Partial deficiency of erythrocyte spectrin in hereditary spherocytosis. www.jci.org /cgi/content/full/99/2/267   (5346 words)

Brain spectrin binding to the NMDA receptor is regulated by phosphorylation, calcium and calmodulin   (Site not responding. Last check: 2007-10-21) Notably, spectrin connects integral membrane proteins to the actin filaments (Morrow, 1989; Bennett and Gilligan, 1993; Davis and Bennett, 1994; Lombardo et al., 1994) and its interactions with the plasma membrane are known to be regulated by calcium, either directly or through calmodulin (CaM) (Steiner et al., 1989; Backman, 1996). The ratios of spectrin binding per amount of protein were calculated for each fragment by densitometry, with the value for the K1086−V1481 fragment normalized to 100% (Figure 3C). To characterize further the association of spectrin with the NMDA-R in situ, rat synaptosomes were pre-incubated with calcium ions prior to solubilization by 1% Triton X-100 and the detergent-soluble fractions were subsequently probed for the presence of spectrin and NMDA-R NR1 and NR2 subunits (Figure 7). www.nature.com /emboj/journal/v17/n14/full/7591095a.html   (6598 words)

Biology : Steven Goodman's Research Interests Ubiquitination of Spectrin Regulates the Erythrocyte Spectrin-Protein-4.1-Actin Ternary Complex Dissociation: Implications for the Sickle Cell Membrane Skleton. Ubiquitination of Erythrocyte Spectrin Regulates the Dissociation of the Spectrin-Adducin-F-Actin Ternary Complex in vitro. Band 3 is a Target Protein of Spectrin’s E2/E3 Activity: Implication for Sickle Cell Disease and Normal Red Blood Cell Aging. www.utdallas.edu /biology/faculty/research/goodman.html   (260 words)

Spectrin and Ankyrin-Based Pathways: Metazoan Inventions for Integrating Cells Into Tissues -- Bennett and Baines 81 ... At the central region of the spectrin tetramers are bound ankyrin/anion exchanger complexes. Members of the spectrin superfamily have in common multiple triple helical repeats, a pair of calponin homology domains at the NH -terminal of one subunit, and a calmodulin-like domain in the COOH-terminal region of one subunit. IV spectrin (24) are colocalized with voltage-gated Na channels at nodes of Ranvier and axon initial segments. physrev.physiology.org /cgi/content/full/81/3/1353   (7685 words)

Spectrin St Claude, a Splicing Mutation of the Human alpha -Spectrin Gene Associated With Severe Poikilocytic Anemia -- ... The membrane skeleton is composed primarily of spectrin tetramers ( Both spectrin chains are primarily composed of repeating units 106 residues in length, which are folded in a coiled-coil triple helical arrangement. The contributions of spectrin to the specific mechanical properties of the erythrocyte membrane skeleton are highlighted by its defects in disorders of red blood cell shape such as hereditary elliptocytosis (HE), hereditary pyropoikilocytosis (HPP), and hereditary spherocytosis (HS). www.bloodjournal.org /cgi/content/full/89/12/4584   (4518 words)

Dynamic molecular modeling of pathogenic mutations in the spectrin self-association domain -- Zhang et al. 98 (6): 1645 ... Spectrin tethers and mesh in the biosynthetic pathway. Low expression allele alpha LELY of red cell spectrin is associated with mutations in exon 40 (alpha V/41 polymorphism) and intron 45 and with partial skipping of exon 46. Gly substitution in helix 1 of beta spectrin repeat 17 that severely disrupts the structure and self-association of the erythrocyte spectrin heterodimer. www.bloodjournal.org /cgi/content/full/98/6/1645   (5091 words)

Drosophila {alpha}- and {beta}-Spectrin Mutations Disrupt Presynaptic Neurotransmitter Release -- Featherstone et al. ... Gelot A, Moreau J, Ben Ari Y, Pollard H (1996) Alpha-brain spectrin mRNA belongs to the population of intradendritically transported mRNAs. Goodman SR (1999) Discovery of nonerythroid spectrin to the demonstration of its key role in synaptic transmission. Wechsler A, Teichberg VI (1998) Brain spectrin binding to the NMDA receptor is regulated by phosphorylation, calcium and calmodulin. www.jneurosci.org /cgi/content/full/21/12/4215   (5575 words)

Association of Spectrin-Like Proteins with the Actin-Organized Aggregate of Endoplasmic Reticulum in the Spitzenkorper ... Spectrin fluorescence reappears in the form of a small patch close to the apical membrane, and later resumes its original position and size in the center of the Spitzenkörper. Baumann O (1998) Association of spectrin with a subcompartment of the endoplasmic reticulum in honeybee photoreceptor cells. Wang Y, Yan L (1991) Immunological identification of spectrins on the plasma membrane of leaf cells of Vicia faba. www.plantphysiol.org /cgi/content/full/125/4/1611   (4949 words)

Yale > Molecular Cellular and Developmental Biology > Graduate Program While many factors contribute, recent evidence indicates that the spectrin based membrane skeleton plays a pivotal role in these processes, beginning with the transport of these proteins through the Golgi, all the way up to and including the control of their organization at the plasma membrane. The targeting of the spectrin skeleton to specific surface membrane domains is being studied in renal epithelial cells and in cultured neurons. We have identified novel forms of spectrin and ankyrin that contribute to a novel vesicular skeleton that appears to mediate protein trafficking from the ER through the Golgi and to the plasma membrane (Devarajan et al.,1996; Devarajan et al.,1997). www.biology.yale.edu /facultystaff/morrow.html   (606 words)

Caenorhabditis elegans {beta}-G Spectrin Is Dispensable for Establishment of Epithelial Polarity, but Essential for ... Levels of ß-G spectrin are low, but are detectable in early embryos (arrowhead indicates four-cell stage), increase after the ~100-cell stage (short arrow), and continue to dramatically increase throughout embryogenesis. Depletion of ß-G spectrin (middle) did not affect localization of MH27 to the adherens junctions at the apicolateral domain of the epidermal epithelia (arrowheads) or the intestinal cells (arrows). the absence of spectrin from the onset of differentiation. www.jcb.org /cgi/content/full/149/4/915   (8379 words)

Organization and dynamics of tryptophan residues in erythroid spectrin: Novel structural features of denatured spectrin ... For fluorescence measurements, the concentration of native spectrin was 0.6 µM, while that of denatured spectrin was 0.4 µM, and the excitation wavelength was 280 nm. The concentration of native spectrin was 0.6 µM, while that of denatured spectrin was 0.4 µM. Spectrin was denatured in 8 M urea. The concentration of spectrin used was 0.1 µM. The excitation wavelength was fixed at 295 nm, and emission was monitored at 337 nm in all cases. www.proteinscience.org /cgi/content/full/12/11/2389   (7684 words)

Spectrin Sequence similarity of the amino-terminal domain of Drosophila beta spectrin to alpha actinin and dystrophin. III spectrin binds to the Arp1subunit of dynactin, J.Biol.Chem. Thomas, G. and Williams, J. Dynamic rearrangement of the spectrin membrane skeleton during the generation of epithelial polarity in Drosophila., J.Cell Sci. www.bms.ed.ac.uk /research/others/smaciver/Encyclop/Abp-s/Spectrin.htm   (529 words)

Brain spectrin binding to the NMDA receptor is regulated by phosphorylation, calcium and calmodulin -- Wechsler and ...   (Site not responding. Last check: 2007-10-21) Brain spectrin binding to the NMDA receptor is regulated by phosphorylation, calcium and calmodulin that the dissociation of spectrin from a phosphorylated NR1 subunit Suzuki, T. and Okumura-Noji, K. NMDA receptor subunits epsilon 1 (NR2A) and epsilon 2 (NR2B) are substrates for Fyn in the postsynaptic density fraction isolated from the rat brain. embojournal.npgjournals.com /cgi/content/full/17/14/3931   (6188 words)

Spectrin It was first discovered in red cells, where it forms part of a lining to the plasma membrane stabilising it against the shear stresses in circulation. We have recently molecularly cloned an isoform of spectrin that is abundant in heart and nervous tissue. As part of our programme, we are collaborating with clinical colleagues to examine the role of spectrin and 4.1 in acute heart failure. www.kent.ac.uk /bio/baines/Research   (405 words)

A New Spectrin, beta IV, Has a Major Truncated Isoform That Associates with Promyelocytic Leukemia Protein Nuclear ... Spectrin is an important component of the membrane skeleton attached to the inner leaf of the lipid bilayer of plasma membranes. Coils A, B, and C of the triple helical coil units of the spectrin-repetitive motifs are indicated. The first residues of the actin/4.1-binding domain, each of the 17 spectrin repeats, and the pleckstrin homology domain are indicated above the sequences with arrowheads. www.jbc.org /cgi/content/full/276/26/23974   (6973 words)

Flexibility of the alpha -Spectrin N-Terminus by EPR and Fluorescence Polarization -- Cherry et al. 79 (1): 526 -- ... Spectrin is a protein underlying the surface of the erythrocyte lipid bilayer. The discrepancy at later positions (especially 49) may be due the larger and more hydrophobic nature of the fluorescent probe compared to the paramagnetic probe, as discussed in more detail in the text. Four different mutations in codon 28 of alpha spectrin are associated with structurally and functionally abnormal spectrin alpha I/74 in hereditary elliptocytosis. www.biophysj.org /cgi/content/full/79/1/526   (5123 words)

Pfam 19.0 : Spectrin   (Site not responding. Last check: 2007-10-21) Spectrin repeats are found in several proteins involved in cytoskeletal structure. The spectrin repeat forms a three helix bundle. Spectrin repeats PUBMED:8266097 are found in several proteins involved incytoskeletal structure. pfam.wustl.edu /cgi-bin/getdesc?acc=PF00435   (203 words)

Stabilities of folding of clustered, two-repeat fragments of spectrin reveal a potential hinge in the human erythroid ... spectrin is unfolded to some degree under physiological conditions. by maleimide to cysteines of spectrin dimers and tetramers in of intact spectrin (41) or fragments thereof (42-44) by atomic www.pnas.org /cgi/content/full/101/6/1502   (4568 words)

Drosophila betaHeavy-spectrin is essential for development and contributes to specific cell fates in the eye -- Thomas ... The spectrin membrane skeleton is a ubiquitous cytoskeletal structure with Dynamic rearrangement of the spectrin membrane skeleton during the generation of epithelial polarity in Drosophila Identification and Characterization of beta V Spectrin, a Mammalian Ortholog of Drosophilabeta H Spectrin dev.biologists.org /cgi/content/abstract/125/11/2125   (845 words)

Welcome to the University of Pennsylvania Institute for Medicine and Engineering Cooperativity in forced unfolding of tandem spectrin repeats. Pathway shifts and thermal softening in temperature-coupled forced unfolding of spectrin domains. Sun A.C, Levitan I. Osmotic stress alters the intracellular distribution of non-erythroidal spectrin (fodrin) in bovine aortic endothelial cells. www.uphs.upenn.edu /ime/publications.html   (2435 words)

Drosophila {alpha}- and {beta}-Spectrin Mutations Disrupt Presynaptic Neurotransmitter Release -- Featherstone et al. ... Spectrins are plasma membrane-associated cytoskeletal proteins implicated in several aspects of synaptic development and function, Contrary to current models, the absence of spectrins {alpha} spectrin is essential for morphogenesis and body wall muscle formation in Caenorhabditis elegans www.jneurosci.org /cgi/content/abstract/21/12/4215   (371 words)

Erythroid Expression of the Human alpha -Spectrin Gene Promoter Is Mediated by GATA-1- and NF-E2-binding Proteins -- ... Spectrin, the most abundant protein of the erythrocyte membrane skeleton, is composed of two structurally similar but nonidentical Friend virus infection, there is marked synthesis of spectrin synthesized spectrin is incorporated into the membrane skeleton www.jbc.org /cgi/content/full/277/44/41563   (4773 words)

Spectrin (Non-Erythroid), TCS, D8B7   (Site not responding. Last check: 2007-10-21) Clone D8B7 - Signet’s anti-human spectrin (Clone D8B7) is developed against the alpha II spectrin SH3 domain (Xu et al., 2001). Clone D8B7 is a specific marker for human, rat and mouse alpha II spectrin or fodrin. It does not cross react with alpha I (erythroid) spectrin SH3 domain. www.signetlabs.com /products/9702.htm   (94 words)

Cooperativity in Forced Unfolding of Tandem Spectrin Repeats -- Law et al. 84 (1): 533 -- Biophysical Journal Cooperativity in Forced Unfolding of Tandem Spectrin Repeats -- Law et al. triple-helical spectrin repeats is often a contiguous helix, in forced unfolding of spectrin is also clearly demonstrated www.biophysj.org /cgi/content/abstract/84/1/533?ct   (457 words)

Spectrin tethers and mesh in the biosynthetic pathway -- De Matteis and Morrow 113 (13): 2331 -- Journal of Cell Science Spectrin tethers and mesh in the biosynthetic pathway -- De Matteis and Morrow 113 (13): 2331 -- Journal of Cell Science recognized ability of spectrin to organize macromolecular complexes of CK2 constitutively associates with and phosphorylates chicken erythroid ankyrin and regulates its ability to bind to spectrin jcs.biologists.org /cgi/content/abstract/113/13/2331   (2123 words)

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