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

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

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

15 5 and 272, as the minimum binding region for beta-spectrin.

16 I spectrin causes profound reductions in all beta spectrins.

17 fficient to mediate protein interaction with beta spectrins.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

40 beta-Spectrin and the membrane skeleton have been propos

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

95 Thus beta spectrin expression in other cells was not required

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

131 +/- 4.0 ( +/- 2 SD) nM, and avidly binds the beta spectrin in MDCK cell lysates.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

147 beta-Spectrin is an erythrocyte membrane protein that is

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

172 In beta-spectrin mutants many axons inappropriately cross t

173 beta-spectrin mutants show specific dose-dependent genet

174 Axons in beta-spectrin mutants spontaneously break.

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

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

177 beta Spectrin mutations were lethal during late embryoni

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

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

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

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

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

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

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

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

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

187 6Arg28Ser peptide showed an affinity for the beta-spectrin peptide comparable to that of Sp alpha 1-1

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

204 13 and 14 (HEalpha13,14) and human erythroid beta-spectrin repeats 8 and 9 (HEbeta8,9), are located o

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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