ライフサイエンスコーパス: spectrin

1 es at the ankyrin- and actin-binding ends of spectrin.

2 not the essential function of non-erythroid spectrin.

3 ions in SPTBN2, a gene that encodes beta-III spectrin.

4 tations in the cytoskeletal protein beta-III-spectrin.

5 those enriched in nodal ankyrinG and betaIV spectrin.

6 consist of diverse beta subunits and alphaII spectrin.

7 Sptan1(f/f) mice for deletion of CNS alphaII spectrin.

8 ctrin causes profound reductions in all beta spectrins.

9 of actins (and other proteins) and edges of spectrins.

10 ent to mediate protein interaction with beta spectrins.

11 igh affinity, resembling other non-erythroid spectrins.

12 tiple isoforms of Nesprin1 (nuclear envelope spectrin 1) that associate with the nuclear envelope (NE

13 form a unique membrane skeleton, composed of spectrin, 4.1R-complex, and ankyrinR-complex components,

14 In this study, we identify spectrin, a contractile protein at the cytoskeleton-memb

15 l and in vitro models have revealed beta-III spectrin, a cytoskeletal protein present throughout the

16 ectromotility, we focused on the betaV giant spectrin, a major component of the outer hair cells' cor

17 e of MSP1 and activates its capacity to bind spectrin, a molecular scaffold protein that is the major

18 a(+) channels and ankyrin G, and then betaIV spectrin, a sequence that reflects the assembly of nodes

19 RIB binds directly to the CH1 domain of beta spectrins, a molecular scaffold that contributes to the

20 In addition, we observed that spectrin, actin, and adducin also form a 2D polygonal la

21 we investigated the spatial organizations of spectrin, actin, and adducin, an actin-capping protein,

22 ne skeleton is a pseudohexagonal meshwork of spectrin, actin, protein 4.1R, ankyrin, and actin-associ

23 hereas it did not affect the organization of spectrin-actin axonal rings imaged by 3D-STORM.

24 inding by SCA5 beta-spectrin interferes with spectrin-actin cytoskeleton dynamics, leading to a loss

25 protein that links membrane proteins to the spectrin-actin cytoskeleton, associates with VE-cadherin

26 ing and bundling protein associated with the spectrin-actin junctions of mature erythrocytes.

27 ilaments function as structural nodes in the spectrin-actin membrane skeleton to optimize the biomech

28 of XK-protein, which is associated with the spectrin-actin-4.1 junctional complex, is associated wit

29 eading to a decrease in its affinity for the spectrin/actin cytoskeleton and causing global membrane

30 Exon 16 of protein 4.1R encodes a spectrin/actin-binding peptide critical for erythrocyte

31 vels, the major peripheral membrane proteins spectrin, adducin, and actin were greatly reduced in FLK

32 depolymerization resistant and sensitive to spectrin, adducin, and nucleator deficiency, consistent

33 used fibroblasts from five patients to study spectrin aggregate formation by Triton-X extraction and

34 Spectrin aggregate formation in fibroblasts with mutatio

35 ble for repression of Yki, while basolateral Spectrins (alpha/beta dimers) are essential.

36 ping Drosophila wing and eye, loss of apical Spectrins (alpha/beta-heavy dimers) produces tissue over

37 biconcave shape of erythrocytes, but whether spectrins also determine the shape of nonerythroid cells

38 However, because spectrins also participate in assembly of axon initial s

39 These data demonstrate that spectrin and actin networks regulate CD44 clustering and

40 6K3 interacts with the cytoskeletal proteins spectrin and adducin whose altered disposition in IP6K3

41 associated with the plasma membrane through spectrin and ank2-L, extend deep into the axoplasm to pr

42 om nodes and axon initial segments of betaIV-spectrin and AnkG mutant mice.

43 beta-spectrin dominantly mislocalizes alpha-spectrin and ankyrin-2, components of the endogenous spe

44 after loss of nodal betaIV spectrin by betaI spectrin and ankyrin-R.

45 As previously described, we show that alpha-Spectrin and beta-Spectrin are essential to maintain a m

46 he tetramerization interaction between alpha-spectrin and beta-spectrins in Drosophila.

47 ges of the highly flexible, dynamic red cell spectrin and effects of a common mutation that disrupts

48 iction-independent cell elongation, as alpha-Spectrin and integrin mutant cells fail to columnarize.

49 Our results identify alpha-Spectrin and integrins as novel regulators of apical con

50 that IQCJ-SCHIP1 also interacts with betaIV-spectrin and Kv7.2/3 channels and self-associates, sugge

51 We conclude that the spectrin and microtubule cytoskeletons work in combinati

52 g the structure of spectrin in situ, the way spectrin and other proteins bind to actin, how the membr

53 dification of the cytoskeletal proteins beta-spectrin and PIEZO1.

54 umans, eliminates detectable binding to beta-spectrin and reduces binding to betaH-spectrin approxima

55 oils and plectonemes in the sensory axons of spectrin and tau double mutants.

56 ical stress and propose that defects in beta-spectrin and tau may sensitize neurons to damage.

57 Degradation of alpha-II spectrin and the expression profiles of caspase-3 and ca

58 elements enriched in Protein 4.1B and betaII spectrin and those enriched in nodal ankyrinG and betaIV

59 Spectrins and actin form a periodic cytoskeleton propose

60 intestinal epithelium of Drosophila, apical Spectrins and Crb are dispensable for repression of Yki,

61 ombining the wormlike chain model for single spectrins and the effective medium theory for the networ

62 cently, an axonal periodic pattern of actin, spectrin, and ankyrin forming 190-nm-spaced, ring-like s

63 Actin, spectrin, and associated molecules form a membrane-assoc

64 Actin, spectrin, and associated molecules form a periodic, subm

65 HHC5/8 with ankyrin-G, ankyrin-G with betaII-spectrin, and betaII-spectrin with phosphoinositides tha

66 hed light on the mechanism by which beta-III-spectrin, and likely similar actin-binding proteins, int

67 ity of three protein model systems: barnase, spectrin, and T4 lysozyme.

68 more closely to the soma, ankyrin G, betaIV-spectrin, and the ion channel expression were maintained

69 istent with all known physical properties of spectrin, and upon full extension our Chinese Finger Tra

70 Cytoskeletal proteins of the axon (betaIV spectrin, ankyrin G) exhibit a high degree of one-dimens

71 betaIV Spectrin, ankyrin G, and, to a lesser extent, the beta1

72 Moreover, a wild-type beta-III spectrin/ankyrin-R complex increases sodium channel leve

73 o beta-spectrin and reduces binding to betaH-spectrin approximately 1000-fold.

74 otably, mutations of SPTNB2 encoding betaIII spectrin are associated with neurodegenerative syndromes

75 cribed, we show that alpha-Spectrin and beta-Spectrin are essential to maintain a monolayered FE, but

76 ein 4.1B and the cytoskeleton protein betaII spectrin are mislocalized in the axon, and assembly of t

77 We also show that ankyrin R and betaI spectrin are not sufficient to prevent nodal disorganiza

78 imary, secondary, and tertiary structures of spectrin are reasonably well defined, but the structural

79 Even though spectrins are essential proteins, alpha-spectrin(R22S) r

80 olayered FE, but, contrary to previous work, spectrins are not required to control proliferation.

81 s regulators of Hippo signalling and suggest Spectrins as potential mechanosensors.

82 indings identify both apical and basolateral Spectrins as regulators of Hippo signalling and suggest

83 CA type-5 whereas homozygous mutations cause spectrin associated autosomal recessive ataxia type-1 (S

84 Spinocerebellar ataxia type 5 (SCA5) and spectrin associated autosomal recessive cerebellar ataxi

85 of spinocerebellar ataxia type-5 (SCA5) and spectrin-associated autosomal recessive cerebellar ataxi

86 yndromes, spinocerebellar ataxia Type 5, and spectrin-associated autosomal recessive cerebellar ataxi

87 ggest that spinocerebellar ataxia Type 5 and spectrin-associated autosomal recessive cerebellar ataxi

88 binding proteins called calmodulin-regulated spectrin-associated protein (CAMSAP)/Patronin/Nezha.

89 periodic cytoskeleton with betaIV and betaII spectrin at nodes of Ranvier and paranodes, respectively

90 is strong evidence that changes in the actin/spectrin-based cortical cytoskeleton of outer hair cells

91 constitutes the major attachment site of the spectrin-based cytoskeleton to the erythrocyte's lipid b

92 of the band 3-ankyrin bridge connecting the spectrin-based cytoskeleton to the membrane.

93 The spectrin-based membrane skeleton maintains the biconcave

94 e thin filaments of striated muscles and the spectrin-based membrane skeleton, use barbed and pointed

95 red cell membrane skeleton is the model for spectrin-based membrane skeletons in all cells, and beca

96 f Na(+)channel clustering is mediated by the spectrin-based paranodal axonal cytoskeleton.

97 previously shown that RhBG is linked to the spectrin-based skeleton through ankyrin-G and that its N

98 like plastin-2 that bundles actin fibers and spectrin beta-chain, brain 1 that links the plasma membr

99 ARCA1) are mirrored in mice lacking beta-III spectrin (beta-III-/-).

100 nic inflammation in wild-type (WT) and beta2-spectrin (beta2SP)(+/-) (SPTBN1) mice.

101 Epigenetic silencing of beta2-spectrin (beta2SP, encoded by SPTBN1), a SMAD adaptor fo

102 of known AIS proteins, including ankyrin G, spectrin betaIV, neurofascin, neuronal cell adhesion mol

103 In frog hair cells, spectrin betaV was invariably detected near the apical j

104 aptive evolution at multiple sites along the spectrin-betaV amino acid sequence in the lineage leadin

105 Interconversion of spectrin between closed dimers, open dimers, and tetrame

106 bly via adducin-mediated inhibition of actin-spectrin binding and cofilin-mediated depolymerization o

107 ously known features of ANK repeats and beta-spectrin-binding activity with a fibrous domain nearly 1

108 insically disordered C-terminal tail of beta-spectrin binds the N-terminal tail of alpha-spectrin, fo

109 Wild-type alpha-spectrin binds to both beta- and betaH-chains with high

110 Apical beta-heavy Spectrin binds to Ex and co-localises with it at the api

111 Two SCA5-associated mutations of beta-III spectrin both reduce ankyrin R levels at the cell membra

112 teolytic neuronal injury biomarkers (alphaII-spectrin breakdown products, SBDPs) and glial cell injur

113 ases with increasing end-to-end distances of spectrins, but has a nonmonotonic dependence on the vari

114 g is also rescued after loss of nodal betaIV spectrin by betaI spectrin and ankyrin-R.

115 easing the dendritic concentration of betaII spectrin by overexpression or by knocking out ankyrin B

116 At paranodes, both axonal proteins (betaII spectrin, Caspr) and glial proteins (neurofascin-155, an

117 n the actin-binding domain (ABD) of beta-III-spectrin causes high-affinity actin binding and decrease

118 of both sexes and found that loss of alphaII spectrin causes profound reductions in all beta spectrin

119 ression of mutant but not wild-type beta-III spectrin causes progressive motor deficits and cerebella

120 nhances and decreases multilayering of alpha-Spectrin cells, respectively.

121 ion volume, enhanced calpain-induced alphaII-spectrin cleavage, and increased cell death in perilesio

122 Ankyrin-G and betaII-spectrin colocalize at sites of cell-cell contact in col

123 ity in cell culture, whereas mutant beta-III spectrin complexes fail to enhance sodium currents.

124 ructure in dendrites, demonstrating that the spectrin concentration is a key determinant in the prefe

125 Using a new alphaII spectrin conditional knock-out mouse, we show that alpha

126 keletal proteins ankyrin-G (AnkG) and betaIV-spectrin control the organization of these complexes and

127 ce microscopy reveals that the length of the spectrin cross-members and the size of the skeletal mesh

128 se results demonstrate the importance of the spectrin cytoskeleton both at the AIS and throughout the

129 Here, we show that the Spectrin cytoskeleton controls Hippo signalling.

130 The spectrin cytoskeleton crosslinks actin to the membrane,

131 imal model has tested the requirement of the spectrin cytoskeleton in maintenance of axon integrity.

132 The spectrin cytoskeleton influences cell spreading and surf

133 uries and diseases because disruption of the spectrin cytoskeleton is a common molecular pathology.

134 The Spectrin cytoskeleton is known to be polarised in epithe

135 Finally, the Spectrin cytoskeleton is required to regulate the locali

136 iption of the entropic elasticity of the RBC spectrin cytoskeleton, including domain unfolding/refold

137 and ankyrin-2, components of the endogenous spectrin cytoskeleton.

138 to organize presynaptic active zones via the spectrin cytoskeleton.

139 Neuronal spectrin cytoskeletons consist of diverse beta subunits

140 s problem and to determine the importance of spectrin cytoskeletons for axon integrity, we generated

141 results demonstrate the broad importance of spectrin cytoskeletons for nervous system function and d

142 ut the nervous system.SIGNIFICANCE STATEMENT Spectrin cytoskeletons play diverse roles in neurons, in

143 alphaII spectrin-deficient mice die before 1 month of age and ha

144 We analyzed alphaII spectrin-deficient mice of both sexes and found that los

145 We analyzed alphaII spectrin-deficient mice of both sexes and found that, in

146 on integrity, we generated mice with alphaII spectrin-deficient peripheral sensory neurons.

147 rain can lead to DAI, and evaluated alpha-II spectrin degradation in the pathophysiology of blast-ind

148 The expression of another alpha-II spectrin degrading enzyme, calpain-2, showed a rapid inc

149 In the absence of betaIII spectrin, dendritic spines collapse onto dendrites.

150 We demonstrate the role of the periodic spectrin-dependent cytoskeleton in axons and show that l

151 ed by large-diameter axons, and that alphaII spectrin-dependent cytoskeletons are also required for a

152 apses, the presynaptic structures in betaIII spectrin-depleted neurons make shaft synapses that exhib

153 odes, respectively, but that loss of alphaII spectrin disrupts this organization.

154 ic flexibility of PRC1, suggests that the MT-spectrin domain interface determines the geometry of the

155 Residues in the spectrin domain of PRC1 contacting the MT are highly con

156 Interestingly, the angle of the spectrin domain on the MT surface corresponds to the pre

157 rant dependence of the unfolded state of the spectrin domain R17 and the intrinsically disordered pro

158 ) on chromosome 2q31.2 in the gene SEC14 and spectrin domains 1 (SESTD1), which encodes a protein inv

159 usly shown that the slow-folding R16 and R17 spectrin domains can be altered to resemble the fast fol

160 Three homologous spectrin domains have remarkably different folding chara

161 structure and sequence to previously studied spectrin domains.

162 We show that SCA5 beta-spectrin dominantly mislocalizes alpha-spectrin and anky

163 We have studied the role of spectrins during epithelia morphogenesis using the Droso

164 EzrA is the founding member of the bacterial spectrin family.

165 defects in the dimer-dimer association of a spectrin filament (as in elliptocytes) cause an even lar

166 that possible attractive forces between the spectrin filaments and the lipid bilayer have on the pre

167 reduced compared to the thermal motion when spectrin filaments are held at equilibrium.

168 It also predicts that because the spectrin filaments are under entropic tension, the therm

169 Lastly, our model predicts that because spectrin filaments are under tension, any axonal injurie

170 ured as a series of actin rings connected by spectrin filaments that are held under tension.

171 he membrane skeleton due to the inability of spectrin filaments to spontaneously form their initial u

172 r tension, any axonal injuries that lacerate spectrin filaments will likely lead to a permanent disru

173 ures that corroborate proposed mechanisms of spectrin flexibility and elasticity.

174 -spectrin binds the N-terminal tail of alpha-spectrin, folding to form the "spectrin tetramer domain"

175 n over 10 months, primarily affecting betaIV spectrin, followed by NaV channels, with modest impact o

176 Spectrins form a submembranous cytoskeleton proposed to

177 Electron microscopy revealed that betaIII spectrin forms a detergent-resistant cytoskeletal networ

178 and found that, in myelinated axons, alphaII spectrin forms a periodic cytoskeleton with betaIV and b

179 leton in axons and show that loss of alphaII spectrin from PNS axons causes preferential degeneration

180 Loss-of beta-III spectrin function appears to underpin cerebellar dysfunc

181 thogenesis mediated through loss of beta-III spectrin function by studying EAAT4 and GLAST knockout m

182 The dominant paradigm for spectrin function is that (alphabeta)2-spectrin tetramer

183 Further elucidation of beta-III spectrin function is therefore needed to understand dise

184 pectrin heterodimer formation and/or alphaII spectrin function.

185 rders appear to result from loss of beta-III spectrin function.

186 We also find that betaII-spectrin functions as a coincidence detector that requir

187 1, but molecular mechanisms linking betaIII spectrin functions to neuronal pathologies remain unreso

188 axonal cytoskeleton consisting of actin and spectrin has been proposed to help axons resist the mech

189 ha) and that mice expressing mutant beta-III spectrin have cerebellar dysfunction with altered mGluR1

190 1 gene, encoding the non-erythrocyte alphaII spectrin, have been associated with severe West syndrome

191 Our results suggest that nodal alphaII spectrin helps resist the mechanical forces experienced

192 3 patients with mutations located within the spectrin heterodimer contact site exhibited severe and p

193 e alpha20 repeat is important for alpha/beta spectrin heterodimer formation and/or alphaII spectrin f

194 ing in the nucleation site of the alpha/beta spectrin heterodimer region.

195 harbouring mutations outside the alpha/beta spectrin heterodimerization domain, four had normal brai

196 ntally-supported quaternary structure of the spectrin heterotetramer.

197 Here, we investigated the organization of spectrin in a variety of neuronal- and glial-cell types.

198 his work identifies a primary role for alpha-Spectrin in controlling cell shape, perhaps by modulatin

199 We show that betaIII spectrin in hippocampal and cortical neurons from rodent

200 ers the mobility and recruitment of beta-III-spectrin in mammalian cells, pointing to a potential dis

201 cal for the polarized distribution of betaII spectrin in neurites.

202 luding questions concerning the structure of spectrin in situ, the way spectrin and other proteins bi

203 results in specific degradation of alpha-II spectrin in the brain along with differential expression

204 alphaII/betaII aggregates and alphaII spectrin in the insoluble protein fraction were observed

205 een shown to cleave the cytoskeletal protein spectrin in vitro.

206 interaction between alpha-spectrin and beta-spectrins in Drosophila.

207 g red cell shape and membrane integrity, and spectrins in other cell types serve these as well as mor

208 e is a correlation between the elongation of spectrins in the cytoskeletal network and the stiffening

209 To determine the role of spectrins in the nervous system, we generated Sptan1(f/f

210 mong axons, but the density of nodal alphaII spectrin increases with axon diameter.

211 that the interaction of alpha-synuclein with spectrin initiates pathological alteration of the actin

212 In summary, we suggest that a functional spectrin-integrin complex is essential to balance adequa

213 microscopy, we show that alphaII and betaIV spectrin interact and form a periodic AIS cytoskeleton.

214 diated pathway is known to inhibit the actin-spectrin interaction in other cell models, we decided to

215 Loss of either DHHC5/8 or ankyrin-G-betaII-spectrin interaction or betaII-spectrin-phosphoinositide

216 We show that endogenous beta-III spectrin interacts with the metabotropic glutamate recep

217 hat high-affinity actin binding by SCA5 beta-spectrin interferes with spectrin-actin cytoskeleton dyn

218 se Finger Trap: at shorter molecular lengths spectrin is a hollow cylinder that extends by increasing

219 nm, our model provides a mechanism by which spectrin is able to undergo a seamless three-fold extens

220 Finally, we found that spectrin is capable of adopting a similar periodic organ

221 We show that the density of nodal betaIV spectrin is constant among axons, but the density of nod

222 Here, we report that beta-III spectrin is essential for the recruitment and maintenanc

223 n which an equivalent mutant Drosophila beta-spectrin is expressed in neurons that extend complex den

224 We show that nodal alphaII spectrin is found at greater densities in large-diameter

225 Although alphaII spectrin is found in neurons in both axonal and somatode

226 copy, which demonstrated that, as predicted, spectrin is hollow at its biological resting length of ~

227 Thus, betaIII spectrin is necessary for formation of the constricted s

228 tional knock-out mouse, we show that alphaII spectrin is required for AIS assembly, neuronal excitabi

229 One function of beta-III spectrin is the stabilization of the Purkinje cell-speci

230 ynaptic terminals is not affected by betaIII spectrin knockdown.

231 to be broadly relevant for the regulation of spectrin-like proteins.

232 dditional membrane-binding domains including spectrin-like repeats (R)1-3, R10-12 and C-terminus (CT)

233 ynthase mu (nNOSmu), which requires specific spectrin-like repeats (SR16/17) in dystrophin's rod doma

234 domain necessary for microtubule binding to spectrin-like repeats 20-22.

235 nteraction of its PDZ domain with dystrophin spectrin-like repeats R16 and R17.

236 arborization coincident with decreased beta-spectrin localization in distal dendrites.

237 oltage-gated sodium (NaV) channel and betaIV spectrin loss with reduced effects on neurofascin 186.

238 eals that restructuring and constraining the spectrin meshwork can fully account for the observed cha

239 Furthermore, spectrin mutant cells show differentiation and polarity

240 of nodes similar to that observed in betaIV-spectrin mutant mice, revealing that IQCJ-SCHIP1 contrib

241 proteins, alpha-spectrin(R22S) rescues alpha-spectrin mutants to adulthood with only minor phenotypes

242 thology of human syndromes caused by betaIII spectrin mutations.

243 ion of demyelinated axons showed that betaIV-spectrin, Nav1.6, and the Kv7.3 channels in nodes of Ran

244 However, in many hematological disorders the spectrin network and lipid bilayer of diseased RBCs may

245 d number of vertical constraints between the spectrin network and the lipid bilayer, which further st

246 odel and predict the extent to which dynamic spectrin network connectivity can protect against failur

247 gher organizational level of the AnkG/betaIV-spectrin network critical for node integrity.

248 shed new light on the formation of the actin-spectrin network in neurons.

249 UB1-processed merozoite surface MSP1 and the spectrin network of the erythrocyte cytoskeleton facilit

250 knobs results in strain inhomogeneity in the spectrin network with elevated shear strain in the knob-

251 hrough its interactions with the AnkG/betaIV-spectrin network.

252 nobs act as structural strengtheners for the spectrin network; on the other, the presence of knobs re

253 requirement for tetramer-based non-erythroid spectrin networks throughout an organism and find that t

254 Ankyrin adaptors together with their spectrin partners coordinate diverse ion channels and ce

255 Here, we show that alphaII spectrin partners with betaIV spectrin to form a periodi

256 yrin-G-betaII-spectrin interaction or betaII-spectrin-phosphoinositide recognition through its plecks

257 We show that a cytoskeletal protein betaIII spectrin plays a key role for the formation of narrow sp

258 onse of our model cytoskeleton, in which the spectrin polymers are treated as entropic springs, is in

259 r linear organization, are homologous to the spectrin proteins that connect actin filaments to the me

260 stress, exploiting mutations in UNC-70 beta-spectrin, PTL-1 tau/MAP2-like and MEC-7 beta-tubulin pro

261 ough spectrins are essential proteins, alpha-spectrin(R22S) rescues alpha-spectrin mutants to adultho

262 on of multiple sites in the N-terminal Sec14/spectrin region of Kal7 may allow coordination of the ma

263 such as Crumbs, Kibra, Expanded, and Merlin, spectrin regulates Hippo signaling in a distinct way by

264 mutations clustered tightly within a single spectrin repeat of DSP cause this novel cardio-cutaneous

265 Abl1 phosphorylated two sites in the fourth spectrin repeat of Kalirin, increasing its sensitivity t

266 that extends by increasing the pitch of each spectrin repeat, which decreases the internal diameter.

267 Nesprins are highly conserved spectrin repeat-containing scaffold proteins predominant

268 Nesprins are a multi-isomeric family of spectrin-repeat (SR) proteins, predominantly known as nu

269 contain a plakin domain formed by up to nine spectrin repeats (SR1-SR9) and an SH3 domain.

270 containing only the Sec14 domain and several spectrin repeats increased spine formation.

271 of Rac GTPase: Abl gates the activity of the spectrin repeats of Trio, allowing them to relieve intra

272 pproximately 90 degree bend between adjacent spectrin repeats.

273 and/or C-helices within each of the mutated spectrin repeats.

274 Knockdown of betaIII spectrin results in a significant decrease in the densit

275 In addition to explaining spectrin's physiological resting length of ~55-65 nm, ou

276 According to the proposed model, spectrin's quaternary structure and mechanism of extensi

277 ide a rationale for how ankyrin-G and betaII-spectrin selectively localize to Madin-Darby canine kidn

278 of active caspase-3, which degrades alpha-II spectrin, showed significant increase in the frontal cor

279 in axons in all neuronal types tested here: Spectrin shows a long-range, periodic distribution throu

280 IQCJ-SCHIP1 binding requires a betaIV-spectrin-specific domain and Kv7 channel 1-5-10 calmodul

281 betaII-Spectrin (SPTBN1) is an adapter protein for Smad3/Smad4

282 gous mutations in the gene encoding beta-III spectrin (SPTBN2) underlie SCA type-5 whereas homozygous

283 drites, we also observed patches of periodic spectrin structures in a small fraction of glial-cell pr

284 llins I-III are members of the alpha-actinin/spectrin subfamily of Dictyostelium calponin homology pr

285 in (ankyrin-R) and its binding partner betaI spectrin substitute for and rescue nodal Na(+) channel c

286 The spectrin superfamily of proteins plays key roles in asse

287 lied to the analysis of a model protein of a spectrin tandem repeat that exemplified an intuitive sta

288 tail of alpha-spectrin, folding to form the "spectrin tetramer domain".

289 from the tetramerization site, destabilizes spectrin tetramers and cell membrane integrity.

290 m for spectrin function is that (alphabeta)2-spectrin tetramers or higher order oligomers form membra

291 al complexes and with edges corresponding to spectrin tetramers such that the edge lengths are given

292 The cytoskeleton consists of spectrin tetramers that are tethered to the lipid bilaye

293 are periodically spaced along the neurite by spectrin tetramers, forming a quasi-1D lattice structure

294 s scaffold of interactions connects beta-III spectrin to a wide variety of proteins implicated in the

295 w that alphaII spectrin partners with betaIV spectrin to form a periodic cytoskeleton at the AIS.

296 r giant AnkG, including recruitment of beta4 spectrin to the AIS that likely is regulated by phosphor

297 nodal Na(+) channels requires axonal betaII spectrin which is concentrated at paranodes.

298 gulated by the local concentration of betaII spectrin, which is higher in axons than in dendrites.

299 nds to the cytoskeletal proteins adducin and spectrin, whose mutual interactions are perturbed in IP6

300 , ankyrin-G with betaII-spectrin, and betaII-spectrin with phosphoinositides that is required for the