ライフサイエンスコーパス: alpha-spectrin

1 15th alpha-helical repeat from chicken brain alpha-spectrin).

2 d, unexpectedly, this role is independent of alpha spectrin.

3 can function independently of an associated alpha spectrin.

4 dimer to KAHRP was inhibited by repeat 4 of alpha spectrin.

5 sists of the first 156 residues of erythroid alpha-spectrin.

6 in a recombinant fragment of human erythroid alpha-spectrin.

7 ncluded the micro-calpain cleavage site from alpha-spectrin.

8 otrypsin inhibitor 2 and the SH3 domain from alpha-spectrin.

9 on of two connected repeats of chicken brain alpha-spectrin.

10 th repeating unit (alpha17) of chicken brain alpha-spectrin.

11 in somatic clones of mutant cells that lack alpha-spectrin.

12 complementary region near the C-terminal of alpha-spectrin.

13 orting this, Ten-m physically interacts with alpha-Spectrin.

14 rin localizes and functions independently of alpha-spectrin.

15 minispectrin contained the shortened sph(1J) alpha-spectrin.

16 behaviour of the R15, R16 and R17 domains of alpha-spectrin.

17 Lu and Lu(v13) was inhibited by repeat 4 of alpha-spectrin.

18 atures in the samples of the SH3 domain from alpha-spectrin.

19 terminus and this activity is independent of alpha-spectrin.

20 r to the crystal structure of the Drosophila alpha-spectrin 14th repeat unit, despite replacement in

21 pha-fetoprotein, stem cell factor, erythroid alpha-spectrin, 2,3-bisphosphoglycerate mutase, insulin-

22 Cleavage of alpha-spectrin, a marker of calpain hyperactivation, is

23 es incorporated the cleavage site present in alpha-spectrin, a naturally occurring substrate of calpa

24 ts 4-11 of beta spectrin with repeats 2-9 of alpha spectrin abolished function but did not prevent po

25 ral variant of alpha-spectrin (L207P) and an alpha-spectrin allele associated with a defect in alpha-

26 Here we examined the effects of two lethal alpha spectrin alleles (alpha-specrg41 and alpha-specrg3

27 identified and analyzed in normal and mutant alpha-spectrin alleles.

28 rst 156 residues in the N-terminal region of alpha-spectrin (alpha N).

29 ar to a corresponding segment from erythroid alpha-spectrin (alphaI) but exhibits unique differences

30 ystal structure of a segment of nonerythroid alpha-spectrin (alphaII) consisting of the first 147 res

31 The structural protein nonerythroid alpha spectrin (alphaIISp) plays a role in the repair of

32 ency in the structural protein, nonerythroid alpha spectrin (alphaIISp), in cells from patients with

33 e previously demonstrated that non-erythroid alpha-spectrin (alphaIISp) is present in mammalian cell

34 entified the structural protein nonerythroid alpha spectrin (alphaSpIISigma) as a component of a nucl

35 While we find that alpha-spectrin also regulates Hippo signaling through Ju

36 nd flexibility of the biologically important alpha-spectrin amino terminal region was examined by the

37 tire alpha and beta spectrin molecules (four alpha spectrin and five beta spectrin fragments), KAHRP

38 kely to form heterodimers and oligomers with alpha spectrin and to interact directly with cellular me

39 deletion of 46 amino acids from repeat 5 of alpha-spectrin and alters spectrin dimer/tetramer stabil

40 Spectrin is required for the localization of alpha-Spectrin and Ankyrin to the postsynaptic membrane.

41 t SCA5 beta-spectrin dominantly mislocalizes alpha-spectrin and ankyrin-2, components of the endogeno

42 As previously described, we show that alpha-Spectrin and beta-Spectrin are essential to mainta

43 We show that both alpha-spectrin and beta-spectrin bind PS and that sites

44 The alpha-spectrin and beta-spectrin genes are composed prim

45 Both alpha-spectrin and beta-spectrin polypeptides consist pr

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

47 d residues connecting pairs of chicken brain alpha-spectrin and human erythroid beta-spectrin repeats

48 constriction-independent cell elongation, as alpha-Spectrin and integrin mutant cells fail to columna

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

50 ization of filamentous Actin, alpha-Tubulin, alpha-Spectrin and nuclear Lamins precede salivary gland

51 The Drosophila genome contains only one alpha-spectrin and one conventional beta-spectrin gene,

52 in caspase 3-cleaved substrates of PARP and alpha-spectrin and subsequent disappearance of caspase-3

53 lasmic structure called a fusome, containing alpha-spectrin and the adducin-like product of the hu-li

54 Alpha-spectrin and the adducin-like protein Hu-li tai sh

55 ere we describe the molecular alterations in alpha-spectrin and their consequences in sph(2BC)/sph(2B

56 o function as heterotetramers comprising two alpha-spectrins and two beta-spectrins.

57 t extracts showed co-sedimentation of xENaC, alpha-spectrin, and Apx.

58 y be important for the efficient assembly of alpha-spectrin, and may reduce its dependence on chapero

59 hila E-cadherin, beta-catenin/Armadillo, and alpha-spectrin, and the disruption of epithelial integri

60 In contrast, erythrocytes from mice lacking alpha-spectrin, ankyrin, protein 4.2, protein 4.1, beta-

61 The anti-alpha-spectrin antibody stained neuronal processes, but

62 dient fractions indicate that ENaC, Apx, and alpha-spectrin are associated in a macromolecular comple

63 d zeta isoforms and proteolytic fragments of alpha-spectrin as proteins released from degenerating ne

64 ical in alphaII, an important difference for alpha-spectrin association with beta-spectrin in forming

65 pecifically to a site near the C terminus of alpha-spectrin at the point that spectrin attaches to ac

66 a showing proteolysis of a calpain substrate alpha-spectrin, autolysis of activated calpain, and redu

67 rometry analysis further showed that band 3, alpha-spectrin, beta-spectrin, and ankyrin were present

68 double-stranded RNA to selectively eliminate alpha-Spectrin, beta-Spectrin, or Ankyrin.

69 viously demonstrated to be essential for the alpha-spectrin:beta-spectrin association of the tetramer

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

71 ing showed that the calpain-specific 145 kDa alpha-spectrin breakdown product (SBDP) increased in hyp

72 lpain 2 and production of a calpain-specific alpha-spectrin breakdown product at 150 kDa were confirm

73 poxic cells, but no caspase-specific 120 kDa alpha-spectrin breakdown product was detected.

74 We have previously demonstrated cleavage of alpha-spectrin by caspase-3 and calpain during apoptosis

75 16th alpha-helical repeat from chicken brain alpha-spectrin) by using the two-domain construct R1516.

76 The adjacent end of alpha-spectrin, called the EF domain, is calmodulin-like

77 The adjacent end of alpha-spectrin, called the EF-domain, is calmodulin-like

78 monstrate that NMJ disassembly after loss of alpha-spectrin can be suppressed by expression of a Wld(

79 Mutations in Drosophila alpha spectrin cause larval lethality and defects in cel

80 ta-spectrin (HEbeta89) and the chicken brain alpha-spectrin (CBalpha1617).

81 he production-defective allele, reticulocyte alpha-spectrin cDNA from one of the original HPP patient

82 hway enhances and decreases multilayering of alpha-Spectrin cells, respectively.

83 both Lu and Lu(v13) bound to repeat 4 of the alpha spectrin chain.

84 hange the amino acid sequence of the encoded alpha-spectrin chain and are not in linkage disequilibri

85 We propose that alpha spectrin combines with the beta-G and beta-H subun

86 onsisting of the first 5 spectrin repeats of alpha-spectrin, comparing normal spectrin with a pathoge

87 results demonstrate that a region 3' of the alpha-spectrin core promoter contains a GATA-1-dependent

88 ientation or position relative to either the alpha-spectrin core promoter or the thymidine kinase pro

89 arction ranges from 70% to 100% in mice with alpha-spectrin deficiency.

90 Cardiac thrombi are present in all adult alpha-spectrin-deficient (sph/sph) mice with severe here

91 either FRAP or SPT analysis were similar for alpha-spectrin-deficient and normal MEL cells, differing

92 FLP recombinase system to generate clones of alpha-spectrin-deficient cells in the ovary, we have sho

93 ected that diffusion of membrane proteins in alpha-spectrin-deficient MEL cells would differ greatly

94 On the alpha-spectrin-deficient MEL cells, the mean diagonal le

95 severe in sph(Dem)/sph(Dem) neonates than in alpha-spectrin-deficient mice with HS.

96 bility complex molecules (MHC) on normal and alpha-spectrin-deficient murine erythroleukemia (MEL) ce

97 Previously, we have shown that infarcts in alpha-spectrin-deficient sph/sph mice become histologica

98 The alpha-spectrin deletion did not interfere with spectrin

99 The data show that the alpha-spectrin EF domain greatly amplifies the function

100 Using pulldown binding assays, we find the alpha-spectrin EF-domain either alone or incorporated in

101 f beta-spectrin binds the N-terminal tail of alpha-spectrin, folding to form the "spectrin tetramer d

102 Six of 7 also exhibited marked increases in alpha-spectrin fragments generated by calpain, a proteas

103 We prepared several recombinant peptides of alpha-spectrin fragments spanning only the Nalpha region

104 Surprisingly, elimination of alpha-spectrin from follicle cells does not appear to pr

105 ned in tandem from the cytoskeletal protein, alpha-spectrin, from chicken brain to ascertain whether

106 compound heterozygote with two mutations in alpha spectrin gene.

107 hylogenetic analysis suggests that the other alpha-spectrin gene (alphaII) common to all vertebrates

108 ue to single nucleotide substitutions in the alpha-spectrin gene coding region that lead to changes i

109 nstrated that the core promoter of the human alpha-spectrin gene directed low levels of erythroid-spe

110 polymorphisms located in this region of the alpha-spectrin gene do not change the amino acid sequenc

111 gion for mutations associated with decreased alpha-spectrin gene expression in patients with heredita

112 ing mechanisms controlling expression of the alpha-spectrin gene is important for understanding eryth

113 ritance of alphaII domain polymorphisms with alpha-spectrin gene mutations causing HE or HPP in Afric

114 on, a given HE/HPP mutation is present in an alpha-spectrin gene of only one haplotype, indicating a

115 ere was complete absence of normally spliced alpha-spectrin gene transcripts derived from a minigene

116 Five polymorphisms of the alpha-spectrin gene, located in a 6-kb interval of genom

117 Five spontaneous, allelic mutations in the alpha-spectrin gene, Spna1, have been identified in mice

118 , sph(2BC), and sph(J), affect the erythroid alpha-spectrin gene, Spna1, on chromosome 1 and cause se

119 ing the erythroid-specific expression of the alpha-spectrin gene.

120 rticle element in intron 10 of the erythroid alpha-spectrin gene.

121 ecause of a mutation in the murine erythroid alpha-spectrin gene.

122 n, caspase-3 activation, and caspase-cleaved alpha-spectrin generation, identical to developmental ne

123 alphaII spectrin (SPTAN1) is one of two alpha spectrin genes and alphaII spectrin dysfunction is

124 Pull-down assays using alpha spectrin GST fusion proteins showed strong associa

125 Soluble nonerythroid alpha-spectrin (half-life=80 min) and beta spectrin (hal

126 and three repeat fragments of chicken brain alpha-spectrin have been determined by X-ray crystallogr

127 30 is a well known structural protein, human alpha spectrin II (alphaSpIISigma*), and that levels of

128 These results demonstrate a role for alpha spectrin in the nucleus as well as a new function

129 ted in a macromolecular complex with Apx and alpha-spectrin in A6 cells and suggest that Apx is requi

130 This work identifies a primary role for alpha-Spectrin in controlling cell shape, perhaps by mod

131 brane skeletal proteins is more dependent on alpha-spectrin in the fusome than at the plasma membrane

132 ysis also revealed the presence of actin and alpha-spectrin in these immunoprecipitates.

133 sph(J)/sph(J) mice synthesize the truncated alpha-spectrin in which the 13-terminal amino acids are

134 e-3 activation (120 kDa breakdown product of alpha-spectrin) in TAI.

135 pe resulting from the loss of the C. elegans alpha spectrin is reproduced by tandem depletion of both

136 Alpha-spectrin is a membrane protein critical for the fl

137 cient cells in the ovary, we have shown that alpha-spectrin is also required for cyst formation and o

138 3-amino acid segment at the COOH-terminus of alpha-spectrin is crucial to the stability of the juncti

139 alpha-Spectrin is dependent upon beta-Spectrin for its n

140 Less clear is whether alpha-spectrin is phosphorylated in vivo and whether spe

141 Furthermore, alpha-spectrin is required for these processes in germli

142 r an allele encoding a structural variant of alpha-spectrin (L207P) and an alpha-spectrin allele asso

143 ereditary elliptocytosis-related mutation of alpha-spectrin, L207P, showed that cell membranes were d

144 Induction of somatic clones lacking alpha-spectrin leads to follicle cell hyperplasia.

145 We suggest that allele alpha spectrin(LEPRA) may be frequently involved in path

146 The maternal allele, designated alpha spectrin(LEPRA), contains transition C-->T in posi

147 4-3-3zeta, pNFH, UCH-L1, and calpain-cleaved alpha-spectrin may serve as a panel of biomarkers with c

148 itary pyropoikilocytosis (HPP) mutations are alpha-spectrin missense mutations in the dimer-tetramer

149 e 3' splice acceptor site, perturbing normal alpha-spectrin mRNA splicing and creating an elongated m

150 defect in cuprophilic cells from labial and alpha spectrin mutants was in morphogenesis of the invag

151 lar localization and/or maintenance, whereas alpha-spectrin mutants exhibit a redistribution of beta-

152 ntial proteins, alpha-spectrin(R22S) rescues alpha-spectrin mutants to adulthood with only minor phen

153 In spc-1 alpha-spectrin mutants, SMA-1 localizes to the apical me

154 Recombinant beta-spectrin C-terminal and alpha-spectrin N-terminal peptides can form tetramer-lik

155 eta-spectrin C-terminal region linked to the alpha-spectrin N-terminal region.

156 Alignment of all available alpha-spectrin N-terminal sequences reveals three blocks

157 tructures to those observed for the isolated alpha-spectrin N-terminal using NMR.

158 in (helix C) in the amino-terminal region of alpha-spectrin (Nalpha region) bundles with another frac

159 ization of filamentous Actin, alpha-Tubulin, alpha-Spectrin, nuclear Lamins and active Caspase 3.

160 action with full-length alpha-spectrin or an alpha-spectrin nucleation site recombinant peptide, alph

161 frequently associated with abnormalities in alpha-spectrin, one of the principal structural proteins

162 bout 230 nM for interaction with full-length alpha-spectrin or an alpha-spectrin nucleation site reco

163 NMJ disassembly despite loss of presynaptic alpha-spectrin or ankyrin2-L.

164 ion in the region spanning residues 17-52 in alpha-spectrin, or that affect hydrophobic clustering an

165 ified functionally important residues in the alpha spectrin partial domain region.

166 served cysteine residue at the C-terminus of alpha-spectrin participates in interactions critical to

167 The association of these alpha-spectrin peptides that have single amino acid repl

168 gest that this particular junction region in alpha-spectrin plays a major role in modulating its asso

169 Mutation in the paternal allele, designated alpha spectrin(PRAGUE), is a transition A to G in the pe

170 slation leading to a significant decrease in alpha spectrin production.

171 -spectrin allele associated with a defect in alpha-spectrin production.

172 hat addition of exon 1' and intron 1' to the alpha-spectrin promoter conferred tissue-specific expres

173 We showed previously that a minimal alpha-spectrin promoter directed low levels of expressio

174 revealed spectrin deficiency and a truncated alpha spectrin protein.

175 frameshift, and production of the truncated alpha spectrin protein.

176 er RNA and consequent decreased synthesis of alpha-spectrin protein are primarily responsible for the

177 5th, 16th, and 17th repeats of chicken brain alpha-spectrin (R15, R16, and R17, respectively) are ver

178 pectrin domains 16 and 17 from chicken brain alpha-spectrin (R16 and R17).

179 analysis of the 16th domain of chicken brain alpha-spectrin, R16.

180 pathway of the 17th domain of chicken brain alpha-spectrin, R17.

181 ven though spectrins are essential proteins, alpha-spectrin(R22S) rescues alpha-spectrin mutants to a

182 All mutant alpha-spectrin recombinant peptides were well folded, st

183 5th, 16th, and 17th domains of chicken brain alpha-spectrin (referred to as R15, R16 and R17, respect

184 imers and confirmed the N-terminal region of alpha-spectrin remains highly flexible in the complex, e

185 ore stably folded fragments, human erythroid alpha-spectrin repeats 1 and 2 (HEalpha1,2) and human er

186 ess stably folded fragments, human erythroid alpha-spectrin repeats 13 and 14 (HEalpha13,14) and huma

187 ats 1 and 2 (HEalpha1,2) and human erythroid alpha-spectrin repeats 2 and 3 (HEalpha2,3), lie nearly

188 ably folded, the fragment of human erythroid alpha-spectrin repeats 4 and 5 (HEalpha4,5) lies opposit

189 ial domain Helix C' of the N-terminal end of alpha-spectrin (residues 14-20 and residues 44-54) in th

190 s at the C- and N-terminal ends of beta- and alpha-spectrin, respectively, on the opposing dimer.

191 We find that the alpha-spectrin segments have, for the most part, evolved

192 experiments on chymotrypsin inhibitor 2 and alpha-spectrin SH3 domain and two circular permutants in

193 ernal PRP and to a circular permutant of the alpha-spectrin SH3 domain by a designed PRP, and bivalen

194 ic data on loop extension mutants of CI2 and alpha-spectrin SH3 domain fit the equation qualitatively

195 The folding/unfolding equilibrium of the alpha-spectrin SH3 domain has been measured by NMR-detec

196 tions of peptides spanning the length of the alpha-spectrin SH3 domain suggested that SH3 domains lac

197 For 33 methyl groups in the alpha-spectrin SH3 domain the average barrier height was

198 les psi in the uniformly (13)C,(15)N-labeled alpha-Spectrin SH3 domain using two different 3D 15N-13C

199 rsion methods on a microcrystalline protein (alpha-spectrin SH3 domain), for which we are able to ide

200 For the case of the 62-residue alpha-spectrin SH3 domain, we determined 13 psi angle co

201 The Lyn and alpha-spectrin SH3 domains exhibited slow, partial unfol

202 ethod is demonstrated here for two proteins, alpha-spectrin SH3 microcrystals and hydrophobin functio

203 n of 46 equivalent C alpha atoms of DtxR and alpha-spectrin SH3 resulted in an rms deviation of 3.0 A

204 In the TS of src SH3 and alpha-spectrin SH3 the distal loop and the associated ha

205 Finally, our work illuminates the results on alpha-spectrin SH3, chymotrypsin inhibitor 2 and beta-la

206 ing in this pathway, thereby identifying the alpha-spectrin SPC-1.

207 permathecal cortex through interactions with alpha-spectrin (SPC-1) and beta-spectrin (UNC-70).

208 d numerous mutations in erythrocyte membrane alpha-spectrin (SPTA1).

209 he Caenorhabditis elegans genome encodes one alpha spectrin subunit, a beta spectrin subunit (beta-G)

210 plectin, another plakin protein, but not in alpha-spectrin, suggesting that the SH3 domain of plakin

211 beta-spectrin tail onto the more structured alpha-spectrin tail.

212 the 14 known HE/HPP mutations located in the alpha-spectrin tetramer binding site.

213 human alphaII-spectrin is closer to fruitfly alpha-spectrin than to human alphaI-spectrin, consistent

214 ructure identifies conformational changes in alpha-spectrin that occur upon binding to beta-spectrin,

215 This is in contrast to mutations in alpha-spectrin, the molecular partner of betaHeavy-spect

216 required for copper cell specification, and alpha-spectrin, thus suggesting an essential role for Gp

217 rin functions independently of an associated alpha spectrin to maintain Na(+) channel clustering at t

218 ivities were confirmed in the degradation of alpha-spectrin to 145 kD spectrin break down product (SB

219 We report that caspase(s) cleave alpha-spectrin to approximately 150-kDa fragments and be

220 caspase cleavage of the cytoskeletal protein alpha-spectrin to approximately 150-kDa fragments is bel

221 SRDs of beta-spectrin proteins interact with alpha-spectrin to form an alpha/beta-spectrin dimer.

222 f beta(H) prevents the stable recruitment of alpha-spectrin to the apical domain, but does not result

223 The nonerythroid alpha spectrin turnover was significantly different (p<0

224 jacent domains from the cytoskeletal protein alpha-spectrin using force profile analysis (FPA).

225 beta-spectrin required for association with alpha-spectrin was determined using recombinant peptides

226 The 62 amino acid SH3 domain from alpha-spectrin was synthesized using the auxiliary-media

227 nemia that results from aberrant splicing of alpha-spectrin, which in turn leads to abnormal erythroc

228 rtial domain helices, show that mutations in alpha-spectrin will affect Helix C' structural flexibili

229 earlier starting point bound to full-length alpha-spectrin with a Kd of about 10 nM, while deletion

230 before the site required for dimerization of alpha-spectrin with beta-spectrin.

231 mias are located at the N-terminal region of alpha-spectrin, with the Arg28 position considered to be

232 tic anemia caused by deficiency of erythroid alpha-spectrin, yet can survive the postnatal period tra