ライフサイエンスコーパス: beta-spectrin

1 exhibit similar association affinities with beta-spectrin.

2 ired for dimerization of alpha-spectrin with beta-spectrin.

3 ie over nearby Z lines and that also contain beta-spectrin.

4 Drosophila mutants lacking either alpha- or beta-spectrin.

5 uantitatively studied their interaction with beta-spectrin.

6 NA repair gene, and SPTBN1, nonerythryocytic beta-spectrin.

7 same altered sarcolemmal arrays that contain beta-spectrin.

8 drin, beta-fodrin, and the muscle isoform of beta-spectrin.

9 caused by a complete deficiency of erythroid beta-spectrin.

10 y either alone or following association with beta-spectrin.

11 n, ZU5A, is directly responsible for binding beta-spectrin.

12 show that alpha-synuclein binds directly to beta-spectrin.

13 phorylation weakens the affinity of 4.1R for beta-spectrin.

14 Actin and 4.1R bind to one end of beta-spectrin.

15 Actin and 4.1 bind to one end of beta-spectrin.

16 5 and 272, as the minimum binding region for beta-spectrin.

17 fficient to mediate protein interaction with beta spectrins.

18 I spectrin causes profound reductions in all beta spectrins.

19 a 77-kDa peptide similar to repeats 10-16 of beta-spectrins.

20 amers comprising two alpha-spectrins and two beta-spectrins.

21 -beta-spectrin and glycophorin C-protein 4.1-beta-spectrin.(1-7) Although evidence supports an essent

22 arry loss-of-function mutations in the mouse beta-spectrin 4 gene (Spnb4) that cause alterations in i

23 at SCRIB binds directly to the CH1 domain of beta spectrins, a molecular scaffold that contributes to

24 show that alpha-synuclein binds directly to beta-spectrin, a key cytoskeletal protein required for l

25 ition, it is known that SRDs neighboring the beta-spectrin ABD enhance actin binding.

26 domain greatly amplifies the function of the beta-spectrin actin-binding domain (ABD) in forming the

27 In the absence of postsynaptic alpha- or beta-Spectrin, active zone size is increased and spacing

28 In these mice, the beta-spectrin Agamma-globin (betasp/Agamma) transgene wa

29 the cytoskeletal proteins, alpha-fodrin and beta-spectrin, also selectively co-immunoprecipitated wi

30 Binding of alpha-synuclein to beta-spectrin alters the organization of the spectrin-an

31 d with a marker for Spnb1 encoding erythroid beta-spectrin, an obvious candidate gene.

32 beta-Spectrin and ankyrin are major components of the me

33 pear to prevent the assembly of conventional beta-spectrin and ankyrin at the lateral domain of the f

34 e associated with the cytoskeletal proteins, beta-spectrin and ankyrin, which may help to maintain th

35 n two additional membrane skeletal proteins: beta-spectrin and ankyrin.

36 observations suggest that interactions with beta-spectrin and ankyrinG help to maintain the concentr

37 ectrin superfamily, including alpha-actinin, beta-spectrin and fimbrin.

38 d bilayer through 2 linkages: band 3-ankyrin-beta-spectrin and glycophorin C-protein 4.1-beta-spectri

39 Antibodies to Ank 2, alpha-fodrin, beta-spectrin and IP(3)R-1 all co-immunoprecipitated NCX

40 pects of regeneration, including the role of beta-spectrin and membrane dynamics, the antagonistic ac

41 ich directly bridges the interaction between beta-spectrin and membrane proteins.

42 ng modification of the cytoskeletal proteins beta-spectrin and PIEZO1.

43 in humans, eliminates detectable binding to beta-spectrin and reduces binding to betaH-spectrin appr

44 aberrant actin binding associated with SCA5 beta-spectrin and spectrin-related disease proteins.

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

46 beta-Spectrin and the membrane skeleton have been propos

47 nning of the first homologous motif for both beta-spectrin and the related dimerization site of alpha

48 associated with decreased phosphorylation of beta-spectrin and with increased phosphorylation of band

49 Neuroglian, ankyrin, alpha beta spectrin, and the Na,K-ATPase colocalize at the lat

50 further showed that band 3, alpha-spectrin, beta-spectrin, and ankyrin were present in a complex wit

51 in alpha-spectrin that occur upon binding to beta-spectrin, and it reports the first structure of the

52 in modulating its association affinity with beta-spectrins, and thus regulates spectrin tetramer lev

53 of GEs with specific peptides in alpha- and beta-spectrin, ankyrin, actin, p55, and protein 4.2.

54 e entrapped ATP derive from sequences within beta-spectrin, ankyrin, band 3, and GAPDH.

55 We prepared anti-beta-spectrin antibodies by using synthetic peptides cor

56 staining was unaffected by preadsorption of beta-spectrin antibodies with A4/beta 1-40 peptide.

57 with these domain-specific affinity purified beta-spectrin antibodies, beta-amyloid plaques were spec

58 -insoluble amyloids were also stained by the beta-spectrin antibodies.

59 It was previously shown that ankyrin and beta spectrin are recruited to sites of cell-cell contac

60 ibit three striking parallels including: (1) beta spectrins are associated with the sites of cell-cel

61 humans, alphaI and alphaII spectrins but not beta spectrins are characterized by the presence of an S

62 y described, we show that alpha-Spectrin and beta-Spectrin are essential to maintain a monolayered FE

63 t is noteworthy that the PS binding sites in beta-spectrin are grouped in close proximity to the site

64 alpha- and beta-Spectrin are major components of a submembrane cyto

65 nor the pleckstrin homology (PH) domains of beta-Spectrin are required for accurate guidance decisio

66 beta-spectrins are crucial for the maintenance of cell s

67 ictly monomers (e.g. dystrophin), alpha- and beta-spectrins are stable as monomeric forms but occur p

68 Spectrin betaIV is the first beta-spectrin associated with a subnuclear structure and

69 The N-terminal domain (residues 1-313) of beta-spectrin associated with F-actin with a Kd of 26 mi

70 rated to be essential for the alpha-spectrin:beta-spectrin association of the tetramerization site.

71 ', the partial domain involved in alpha- and beta-spectrin association, exhibits little interaction w

72 ATPase, codistributes with ankyrin and alpha beta spectrin at sites of neuroglian-mediated contact.

73 association of human erythrocytes alpha and beta spectrin at the tetramerization site involves inter

74 essed in mdx muscle, also codistributes with beta-spectrin at the mutant sarcolemma.

75 d in desmin -/- muscle and redistribute with beta-spectrin at the sarcolemma when costameres are lost

76 ating the association affinity of alpha- and beta-spectrin at the tetramerization site of different i

77 pha-catenin and the amino-terminal domain of beta-spectrin augment the interaction between alpha-cate

78 tide, consisting of the C-terminal region of beta-spectrin (beta C), was determined, and structural c

79 The non-pleckstrin homology domain beta-spectrin (beta2SP) (the official name for human is

80 We report that beta-spectrin (betaI) replaces beta-fodrin (betaII) at t

81 a-fodrin (alphaII) and the muscle isoform of beta-spectrin (betaISigma2).

82 erall similarity of 89.0% and 95.3% to mouse beta-spectrin (betaSpIIsigma1) at the nucleotide and ami

83 We show that both alpha-spectrin and beta-spectrin bind PS and that sites of high affinity ar

84 previously known features of ANK repeats and beta-spectrin-binding activity with a fibrous domain nea

85 intrinsically disordered C-terminal tail of beta-spectrin binds the N-terminal tail of alpha-spectri

86 ss-dependent labeling kinetics of alpha- and beta-spectrin by LC-MS/MS identifies Cys in these antipa

87 Recombinant beta-spectrin C-terminal and alpha-spectrin N-terminal p

88 sence and presence of a model protein of the beta-spectrin C-terminal end.

89 a fused "mini-spectrin dimer" containing the beta-spectrin C-terminal region linked to the alpha-spec

90 Deficiencies of either alpha- or beta-spectrin can result in severe hereditary spherocyto

91 al domain in the carboxyl-terminal region of beta-spectrin (Cbeta region) to yield a triple alpha-hel

92 -containing liposomes with native alpha- and beta-spectrin chains and with recombinant spectrin fragm

93 s obtained if and only if Zu5 was mixed with beta-spectrin constructs containing repeats 14 and 15 in

94 The results suggest that beta-Spectrin contributes to midline repulsion through t

95 ncoupling of complementary SRDs in the alpha/beta-spectrin dimer.

96 nteract with alpha-spectrin to form an alpha/beta-spectrin dimer.

97 We show that SCA5 beta-spectrin dominantly mislocalizes alpha-spectrin and

98 For short constructs of alpha- and beta-spectrin, either as monomers or as alpha,beta-dimer

99 ral repeat, which binds to the complementary beta-spectrin element, and the adjacent complete repeat

100 lt, we have cloned three isoforms of a novel beta-spectrin elf (embryonic liver beta-fodrin), and her

101 We mapped the 5'-end of the beta-spectrin erythroid cDNA and cloned the 5'-flanking

102 s of nematode beta-G spectrin and vertebrate beta spectrins exhibit three striking parallels includin

103 Thus beta spectrin expression in other cells was not required

104 f a beta spectrin transgene or to knock down beta spectrin expression with dsRNA.

105 ed remodeling of alphaI spectrin and altered beta-spectrin expression and localization.

106 onal expression of an N-terminally truncated beta-spectrin fails to rescue lethality resulting from a

107 teraction between members of the ankyrin and beta-spectrin families previously established in erythro

108 rin is the generally expressed member of the beta-spectrin family of elongated polypeptides that form

109 alpha-Spectrin is dependent upon beta-Spectrin for its normal subcellular localization an

110 trin molecules (four alpha spectrin and five beta spectrin fragments), KAHRP bound only to one, the a

111 ance yielded a K D of 15.2 nM for binding of beta-spectrin fragments to the ankyrin subdomain Zu5, ac

112 hted by its evolutionary conservation in all beta spectrins from Drosophila to humans.

113 on, we identified the adaptor protein ELF, a beta-spectrin from stem/progenitor cells committed to fo

114 re undertaken to identify sites of essential beta spectrin function in Drosophila and to determine wh

115 establish that DAnk2-binding is critical for beta spectrin function in vivo.

116 These defects are consistent with beta-spectrin function in anchoring proteins at cell mem

117 dicating that the N-terminus is essential to beta-spectrin function in vivo.

118 pectrin occur in the nervous system; and (3) beta spectrin-G in striated muscle is associated with po

119 me polymorphisms of the coding region of the beta-spectrin gene are also described.

120 g analyses, and gel mobility shift assays, a beta-spectrin gene erythroid promoter with two binding s

121 We have discovered another member of the beta-spectrin gene family by homology searches of the Ge

122 mutations and four missense mutations of the beta-spectrin gene in 11 unrelated families.

123 re required for high level expression of the beta-spectrin gene in these tissues.

124 se findings underscore the importance of the beta-spectrin gene mutations in the pathogenesis of HS a

125 rding spectrin, only three isolated cases of beta-spectrin gene mutations were recently reported in a

126 The beta-spectrin gene promoter was able to be transactivate

127 flanking genomic DNA containing the putative beta-spectrin gene promoter.

128 We have screened the coding region of the beta-spectrin gene using the SSCP technique, in 40 famil

129 only one alpha-spectrin and one conventional beta-spectrin gene, making it an ideal system to genetic

130 ermine the tissue-specific expression of the beta-spectrin gene.

131 The alpha-spectrin and beta-spectrin genes are composed primarily of tandemly r

132 Four mammalian beta-spectrin genes are currently recognized, all encode

133 ross the repeated segments of the alpha- and beta-spectrin genes.

134 throid alpha-spectrin (half-life=80 min) and beta spectrin (half-life=53 min) turn over more slowly t

135 ard to the first group, only one mutation of beta spectrin has been reported in the literature.

136 plicing of pre-mRNA transcripts of alpha and beta spectrin has emerged as an important generator of d

137 Remarkably, mice lacking nodal beta spectrins have normal nodal Na(+) channel clusterin

138 eat units (DSRUs) from the human erythrocyte beta-spectrin (HEbeta89) and the chicken brain alpha-spe

139 in the alpha20 repeat is important for alpha/beta spectrin heterodimer formation and/or alphaII spect

140 falling in the nucleation site of the alpha/beta spectrin heterodimer region.

141 ients harbouring mutations outside the alpha/beta spectrin heterodimerization domain, four had normal

142 Tetramers of alpha- and beta-spectrin heterodimers, linked by intermediary prote

143 The results establish a role for beta spectrin in determining the subcellular distributio

144 +/- 4.0 ( +/- 2 SD) nM, and avidly binds the beta spectrin in MDCK cell lysates.

145 The results show that 1) overexpression of beta spectrin in most of the cell types studied was leth

146 ll types studied was lethal; 2) knockdown of beta spectrin in most tissues had no detectable effect o

147 an unanticipated role of the first repeat of beta-spectrin in actin binding activity and of the secon

148 fference for alpha-spectrin association with beta-spectrin in forming tetramers.

149 restingly, the complete absence of erythroid beta-spectrin in jaundiced mice leads to no detectable s

150 cross the CNS midline, suggesting a role for beta-Spectrin in midline repulsion.

151 er steady-state level than either ankyrin or beta-spectrin in mouse fetal liver cells.

152 The presence of beta-spectrin in the amyloid plaques in a subset of spor

153 ation interaction between alpha-spectrin and beta-spectrins in Drosophila.

154 were very important in its association with beta-spectrin, in the following order: L49 > G46 > K48.

155 e other measurements except for nonerythroid beta spectrin, indicating that these subunits are protec

156 est that high-affinity actin binding by SCA5 beta-spectrin interferes with spectrin-actin cytoskeleto

157 The critical domains of beta-spectrin involved in complex formation were determi

158 beta-Spectrin is a major component of the membrane skele

159 in labeled transverse sections reveals that beta-spectrin is also concentrated in perijunctional reg

160 beta-Spectrin is an erythrocyte membrane protein that is

161 clein-related disorders, we demonstrate that beta-spectrin is critical for alpha-synuclein neurotoxic

162 that the Caenorhabditis elegans homologue of beta-spectrin is encoded by the unc-70 gene.

163 del in which an equivalent mutant Drosophila beta-spectrin is expressed in neurons that extend comple

164 apical plasma membrane of epithelial cells; beta-spectrin is found at the lateral membrane.

165 beta-Spectrin is normally found at the sarcolemma in cos

166 Thus, beta-spectrin is not essential for general membrane inte

167 Double antibody labeling shows that beta-spectrin is precisely colocalized with both VGSCs a

168 letal muscle, using immunofluorescence, that beta-spectrin is precisely colocalized with both VGSCs a

169 However, beta-spectrin is required for a subset of processes at c

170 Further, the ankyrin binding domain of beta-spectrin is required for alpha-synuclein binding an

171 Mechanistically, beta-Spectrin is required for the localization of alpha-

172 In this study, we demonstrate that beta-spectrin is required for the physical integrity of

173 Here, we demonstrate that beta-Spectrin is required in neurons for proper midline

174 ized the synapsin I attachment site upon the beta-spectrin isoform betaSpIISigmaI to a region of 25 a

175 encodes a homolog of betaH-spectrin, a novel beta-spectrin isoform first identified in Drosophila.

176 First, we tested mutant beta-spectrins lacking ankyrin binding activity and/or t

177 In neurons, the loss of beta-spectrin leads to abnormal axon outgrowth.

178 In muscles, a loss of beta-spectrin leads to disorganization of the myofilamen

179 Disruption of the adaptor protein ELF, a beta-spectrin, leads to disruption of transforming growt

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

181 n fails to rescue lethality resulting from a beta-spectrin loss-of-function allele, indicating that t

182 t have single amino acid replacements with a beta-spectrin model peptide, consisting of the C-termina

183 ortance of the regulatory role played by the beta spectrin molecule in the assembly of alphabeta spec

184 gments, which encompass the entire alpha and beta spectrin molecules (four alpha spectrin and five be

185 nd ankyrin were not significantly altered in beta spectrin mutants, indicating that the two isoforms

186 defective motor axon regeneration in unc-70/beta-spectrin mutants and a candidate gene screen.

187 Protein null alpha- and beta-spectrin mutants are embryonic lethal and display s

188 In beta-spectrin mutants many axons inappropriately cross t

189 beta-spectrin mutants show specific dose-dependent genet

190 Axons in beta-spectrin mutants spontaneously break.

191 overcome the lethality of a loss-of-function beta spectrin mutation.

192 In contrast, beta spectrin mutations had a striking effect on the bas

193 beta Spectrin mutations were lethal during late embryoni

194 Quantitative evidence is presented that the beta-spectrin N-terminal domain plus the first two alpha

195 igated in Drosophila the significance of the beta-spectrin N-terminus, and explored its functional in

196 autosomal dominant inheritance of one of the beta-spectrin null mutations.

197 omatography revealed that phosphorylation of beta-spectrin occurs in a sequential manner where each s

198 Phosphorylation of beta-spectrin on Ser/Thr is well recognized.

199 , oncoretroviral vectors containing either a beta-spectrin or beta-globin promoter and the alpha-glob

200 Activated PKC and the PH domain of beta-spectrin or dynamin-1 concomitantly bound to RACK1.

201 RNA to selectively eliminate alpha-Spectrin, beta-Spectrin, or Ankyrin.

202 e found that embryonic liver fodrin (ELF), a beta-Spectrin originally identified in endodermal stem/p

203 A four-motif beta-spectrin peptide (beta1-4+) with this earlier start

204 6Arg28Ser peptide showed an affinity for the beta-spectrin peptide comparable to that of Sp alpha 1-1

205 sured by the enhanced competitive entry of a beta-spectrin peptide possessing both actin- and 4.1R-bi

206 ough these 2 mutant peptides associated with beta-spectrin peptide with significantly differing affin

207 itated entry into the junctions in situ of a beta-spectrin peptide, containing the actin- and 4.1R-bi

208 ) for construction of dimerization competent beta-spectrin peptides was particularly critical.

209 The oxysterol binding protein and beta-spectrin PH domains bound PtdIns-3,4,5-P3 and PtdIn

210 ELF, a beta-spectrin, plays a key role in the transmission of T

211 Both alpha-spectrin and beta-spectrin polypeptides consist primarily of triple c

212 the coiled-coil domain is also restricted to beta-spectrin-positive puncta, while the isolated spectr

213 cement of the gamma-globin promoter with the beta-spectrin promoter allows the expression of the beta

214 sgenic mice, a reporter gene directed by the beta-spectrin promoter was expressed in erythroid tissue

215 ed to a 576-bp fragment containing the human beta-spectrin promoter.

216 that express human beta-III-spectrin or fly beta-spectrin proteins containing SCA5 mutations.

217 It is known that the SRDs of beta-spectrin proteins interact with alpha-spectrin to f

218 nical stress, exploiting mutations in UNC-70 beta-spectrin, PTL-1 tau/MAP2-like and MEC-7 beta-tubuli

219 able of recognizing the C-terminal region of beta-spectrin regardless of its phosphorylation state an

220 In contrast, human erythroid beta-spectrin repeats 13, 14, 15, and 16 (prepared in al

221 affinity pull-down assays implicated Zu5 and beta-spectrin repeats 14-15 as the minimum binding epito

222 13 and 14 (HEalpha13,14) and human erythroid beta-spectrin repeats 8 and 9 (HEbeta8,9), are located o

223 ken brain alpha-spectrin and human erythroid beta-spectrin repeats can undergo bending without losing

224 tively, these results show the 14th and 15th beta-spectrin repeats comprise the minimal, phased regio

225 subdomains are replaced with the stretchable beta-spectrin repeats, we show that the stretchable prop

226 In this study, the minimal region of beta-spectrin required for association with alpha-spectr

227 dentification of the specific phosphorylated beta-spectrin residues and the ordered sequence of phosp

228 In the absence of beta-spectrin, residues 14-20 and 46-52 were known to be

229 Pathogenic mutations in alpha and beta spectrin result in a variety of syndromes, includin

230 as a deficiency of human erythroid alpha- or beta-spectrin results in hereditary spherocytosis (HS).

231 9 (goliath, E3 ubiquitin ligase), or SPTBN1 (beta-Spectrin, scaffolding protein) caused synergistic h

232 le considerable heterogeneity is found among beta-spectrin segments.

233 Using comparative genomics, erythroid beta-spectrin (sptb) was identified as the gene mutated

234 -amyloid plaques, and that an abnormality of beta-spectrin structure or function may be involved in t

235 genome encodes one alpha spectrin subunit, a beta spectrin subunit (beta-G), and a beta-H spectrin su

236 It remains unclear as to whether novel beta-spectrins such as elf1-4 are distinct genes or beta

237 ) changed dramatically upon association with beta-spectrin, suggesting that the two regions undergo a

238 the docking of the intrinsically disordered beta-spectrin tail onto the more structured alpha-spectr

239 However, in the skeletal muscle of mdx mice, beta-spectrin tends to be absent from the sarcolemma ove

240 n, and it reports the first structure of the beta-spectrin tetramerization domain.

241 ficant increase in phosphorylation levels of beta-spectrin that is inhibited by preincubation of RBCs

242 butions of four specific functional sites in beta spectrin to its assembly and function.

243 mbinant spectrin peptides to model alpha and beta spectrin to study their association at the tetramer

244 ctrin to approximately 150-kDa fragments and beta-spectrin to approximately 120- and approximately 80

245 spectrin mutants exhibit a redistribution of beta-Spectrin to the axon scaffold.

246 ice site of intron 17 leading to an aberrant beta spectrin transcriptional message lacking exons 16 a

247 to drive tissue-specific overexpression of a beta spectrin transgene or to knock down beta spectrin e

248 nervous system-specific expression of a UAS-beta spectrin transgene was sufficient to overcome the l

249 interactions with alpha-spectrin (SPC-1) and beta-spectrin (UNC-70).

250 the putative helical bundling in alpha- and beta-spectrin undoubtedly play a significant role in tet

251 to the N-terminal and C-terminal domains of beta-spectrin variants.

252 In contrast, beta-spectrin, VGSCs, and ankyrinG have a punctate distr

253 dystrophin, beta-dystroglycan and a form of beta-spectrin was strong but that for utrophin was marke

254 A 289-kDa isoform, similar to full-length beta-spectrins, was partially assembled from sequences i

255 system is the association between alpha- and beta-spectrin, where two partially structured, incomplet

256 membrane skeleton is composed of alpha- and beta-spectrin, which associate to form heterodimers and

257 peats comprise the minimal, phased region of beta-spectrin, which binds ankyrin at the Zu5 subdomain

258 ) Replacement of repetitive segments 4-11 of beta spectrin with repeats 2-9 of alpha spectrin abolish

259 ophila and Caenorhabditis elegans, a variant beta spectrin with unusual properties has been recognize

260 of a specific region near the N-terminal of beta-spectrin with a complementary region near the C-ter

261 hemistry revealed partial co-localization of beta-spectrin with NCX1, Na(+) pump alpha3, and IP(3)R-1