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

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

5 orting this, Ten-m physically interacts with 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 minispectrin contained the shortened sph(1J) alpha-spectrin.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

70 16th alpha-helical repeat from chicken brain alpha-spectrin) by using the two-domain construct R1516.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

93 The alpha-spectrin deletion did not interfere with spectrin

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

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

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

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

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

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

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

101 compound heterozygote with two mutations in alpha spectrin gene.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

121 30 is a well known structural protein, human alpha spectrin II (alphaSpIISigma*), and that levels of

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

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

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

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

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

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

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

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

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

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

132 3-amino acid segment at the COOH-terminus of alpha-spectrin is crucial to the stability of the juncti

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

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

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

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

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

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

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

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

141 4-3-3zeta, pNFH, UCH-L1, and calpain-cleaved alpha-spectrin may serve as a panel of biomarkers with c

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

167 revealed spectrin deficiency and a truncated alpha spectrin protein.

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

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

170 5th, 16th, and 17th repeats of chicken brain alpha-spectrin (R15, R16, and R17, respectively) are ver

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

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

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

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

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

176 5th, 16th, and 17th domains of chicken brain alpha-spectrin (referred to as R15, R16 and R17, respect

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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