ライフサイエンスコーパス: gamma-crystallin

1 be efficient to inhibit aggregation of lens gamma-crystallin.

2 6, alphaA-crystallin, alphaB-crystallin, and gamma-crystallin.

3 three major crystallins: alpha-, beta-, and gamma-crystallin.

4 entiation and nearly abolished expression of gamma-crystallin.

5 st high-resolution X-ray structures of human gamma crystallins.

6 the aggregation and phase separation of the gamma crystallins.

7 c reduction in expression of their beta- and gamma-crystallins.

8 N showed significant expression of beta- and gamma-crystallins.

9 ractions also exist among alpha-, beta-, and gamma-crystallins.

10 aining the solubility of denatured beta- and gamma-crystallins.

11 in orientation is different from native beta gamma-crystallins.

12 as a result of an almost complete absence of gamma-crystallins.

13 ot significantly with antibodies to beta- or gamma-crystallins.

14 ax6, c-Maf, E-cadherin and alpha-, beta- and gamma-crystallins.

15 tallin genes but low expression of beta- and gamma-crystallins.

16 ract, which is caused by an R58H mutation in gammaD crystallin.

17 face crucial for unusually high stability of gammaD-crystallin.

18 gregation propensity of the eye-lens protein gammaS-crystallin.

19 een associated with a G18V mutation in human gammaS-crystallin.

20 s operating in a different crystallin, human gammaS-crystallin.

21 ospray ionization mass spectra of human lens gammaS-crystallins.

22 control experiments on in vitro alphaB- and gammaD-crystallin, 2D IR spectroscopy can identify the h

23 gamma-Crystallins A through F also became insoluble duri

24 The cysteine residues of the gamma crystallins, a family of ocular lens proteins, are

25 e individual unfolding pathways of the human gammaD-crystallin, a multidomain protein that must remai

26 Immunohistochemistry data revealed gamma-crystallin aggregates at the cell boundaries of in

27 Intranuclear gamma-crystallin aggregates, incomplete denucleation, an

28 activity was assessed through inhibition of gamma-crystallin aggregation induced by singlet oxygen.

29 Mutant gammaB-S11R proteins triggered the gamma-crystallin aggregation that probably disrupted mem

30 as assayed by luciferase refolding and human gammaD-crystallin aggregation suppression and refolding.

31 , we propose an initial structural model for gammaD-crystallin amyloid fibrils.

32 In contrast, gamma-crystallin and actin interacted with MBP-alphaB at

33 creased the scattering of light by beta- and gamma-crystallin and alcohol dehydrogenase.

34 hese peptides with intact alpha-, beta-, and gamma-crystallins and alcohol dehydrogenase, a protein u

35 the highly unusual amino acid composition of gamma-crystallins and their functional homologues.

36 betaB1, betaB2, betaB3, gammaS, gammaC, and gammaD-crystallins and mapped their positions on two-dim

37 rtant because it blocks disulfide bonding of gammaS-crystallins and, thereby, inhibits formation of t

38 teristics of bound substrates (rhodanese and gamma-crystallin) and the results of fluorescence quench

39 nker, each containing a proline conserved in gamma-crystallins, and the resulting steric constraints

40 atural lens proteins, beta(H) crystallin and gammaD crystallin, and in vitro chaperone target protein

41 ion defects, including reduced expression of gamma-crystallins; and cataract formation.

42 MIP did not interact with gammaD-crystallin, another member of the highly conserve

43 s performed using an antibody to bovine lens gamma-crystallins, applied to protein extracts in immuno

44 Human gamma-crystallins are long-lived, unusually stable prote

45 gamma-Crystallins are major components of the dense, hig

46 Beta- and gamma-crystallins are major protein constituents of the

47 gamma-Crystallins are major structural components of the

48 A number of point mutations in gammaD-crystallin are associated with human cataract.

49 68, 156 in human gammaD- and Trp72 in human gammaS-Crystallin are buried, but water can reach amide

50 BetaB1- and gammaS-crystallins are normally abundant in adult mammal

51 Proteomics identified gamma-crystallin as a protein that was substantially dim

52 Further analysis of the modified gammaS-crystallin as tryptic peptides located the modifi

53 Transgenic expression of mutant CRYGC5bpd gamma-crystallin at near-physiological levels causes len

54 resulting in amino acid substitutions in the gamma-crystallin buried cores (two in the N-terminal dom

55 These results further suggest that HCG5pbd gamma-crystallin causes cataracts through a direct toxic

56 S mutation in the N-terminal domain of mouse gammaS-crystallin causes the severe Opj cataract, with d

57 Human gammaS-crystallin cDNA was cloned into pET-20b(+), and t

58 ibitor, E-64, blocked cataract formation and gamma-crystallin cleavage in alpha 3 (-/-) lenses.

59 ed in, or part of the pathway that leads to, gamma-crystallin cleavage is presented.

60 ers of the betagamma-crystallin superfamily, gammaS-crystallin comprises two similar beta-sheet domai

61 gamma-Crystallins constitute the major protein component

62 o assess mRNA levels for CP49, filensin, and gammaS-crystallin (control).

63 t they are hitherto novel proteins with beta/gamma-crystallin domains, cysteine-rich regions and pote

64 rkers for differentiation, such as beta- and gamma-crystallins, even though the cells do not withdraw

65 00, failed to upregulate beta-crystallin and gamma-crystallin expression.

66 linking product between Cys-18 and Cys-78 in gamma-crystallin F was identified by matrix-assisted las

67 he sulfhydryl groups of Cys-18 and Cys-78 in gamma-crystallin F, which are within a distance of 3.57A

68 malian lens, the expression of the beta- and gamma-crystallin families is thought to be limited to fi

69 rkers including alphaA-, alphaB-, beta-, and gamma-crystallins, filensin, CP49, and MIP/aquaporin 0.

70 mmaS with the results obtained for the other gamma-crystallins for which the critical temperature is

71 The oxidation-mimicking W42Q mutant of gammad-crystallin formed non-native polymers starting fr

72 beta gamma-Crystallins from the eye lens are proteins consist

73 ration (LLPS) of aqueous solutions of bovine gammaD-crystallin (gammaD), a protein in the eye lens.

74 The three major human lens monomeric gamma-crystallins, gammaD, gammaC, and gammaS, all refol

75 gammaS-crystallin (gammaS) is an important human and bov

76 In the human eye lens, gammaS-crystallin (gammaS-WT) forms a densely packed tra

77 taracts have been linked to mutations in the gamma crystallin gene.

78 mutation (Cryge(Cat2-Elo)), a member of the gamma-crystallin gene cluster (Cryg).

79 indred revealed linkage at 2q33-35 where the gamma-crystallin gene cluster resides.

80 s and mice have been shown to map within the gamma-crystallin gene cluster.

81 llin, another member of the highly conserved gamma-crystallin gene family.

82 en linked recently to point mutations in the gammaD crystallin gene.

83 ported to have a point mutation in the human gammaD crystallin gene.

84 mice were caused by a point mutation in the gammaD-crystallin gene (gammaD-V76D).

85 The P23T mutation in the human gammaD-crystallin gene has in recent years been associat

86 Mutations in the human gammaD-crystallin gene have been linked to several types

87 t cataract caused by a point mutation in the gammaD-crystallin gene.

88 and spatial regulation of alphaB, alphaA and gamma-crystallin genes in mouse embryonic lens by using

89 ein with a promoter element conserved in all gamma-crystallin genes is responsible for their expressi

90 transgene applications using an abbreviated gamma-crystallin GFP cassette.

91 r, the Pro23 to Thr (P23T) mutation of human gammaD crystallin has been linked to cerulean, lamellar,

92 structure of the C-terminal domain of human gammaS-crystallin has been solved at 2.4 A resolution.

93 We show that all lens gamma-crystallins have evolved a significantly elevated

94 Because the six murine gamma-crystallins have generally been regarded as specif

95 spectra of single Trps in human gammaD- and gammaS-Crystallins have been measured with both an upcon

96 Human gammaD-crystallin (HgammaD-Crys) is a monomeric, two-dom

97 Human gammaD-crystallin (HgammaD-Crys) is a two-domain beta-sh

98 Human gammaD-crystallin (HgammaD-Crys) is a two-domain, beta-s

99 Human gammaD-crystallin (HgammaD-Crys) is a very stable eye le

100 e have expressed recombinant wild-type human gammaD crystallin (HGD) and its Arg-14 to Cys mutant (R1

101 Several point mutations in human gammaD-crystallin (HGD) are now known to be associated w

102 he Arg14 to Cys (R14C) mutation in the human gammaD-crystallin (HGD) gene has been associated with a

103 ciated Pro23 to Thr (P23T) mutation in human gammaD-crystallin (HGD) has a variety of phenotypes and

104 The P23T mutant of human gammaD-crystallin (HGD) is associated with cataract.

105 caused by this mutation, we expressed human gammaD-crystallin (HGD), the P23T mutant, and other rela

106 GammaS-crystallin, important in maintaining lens transpa

107 ed beta-sheet Greek key domains of beta- and gamma-crystallins in humans and all other vertebrates ea

108 tructural role of MIP in the organization of gamma-crystallins in lens fibers.

109 The expression of gamma-crystallins in the developing murine retina sugges

110 Amorphous aggregation of gamma-crystallins in the eye lens causes cataract, a wid

111 erized by upregulation of alpha-, beta-, and gamma-crystallins in the retina.

112 ited by the accumulation of UV-damaged human gammaD-crystallins in the eye lens.

113 ificant cross-linking as well as cleavage of gammaS-crystallin in all adult lenses.

114 The core function of gammaS-crystallin in the eye lens may be precisely its c

115 tructures of the two-domain proteins L11 and gammaD-Crystallin, in which the linkers between the doma

116 lens phenotype, including solubilization of gamma-crystallin, increased lens transparency and induct

117 upports the contention that S-methylation of gammaS-crystallin inhibits protein insolubilization and

118 The mutant gamma-crystallins initially disrupt nuclear function, bu

119 t scattering due to altered homologous gamma-gamma crystallin interactions.

120 The structural eye lens protein gammaD-crystallin is a major component of cataracts, but

121 Human gammaD-crystallin is an eye lens protein that aggregates

122 The V75D mutant of gammaD-crystallin is associated with congenital cataract

123 r dynamics simulations that the stability of gammaD-crystallin is greatly reduced by the conversion o

124 This work also suggests that gammaD-crystallin is one of the crucial components for t

125 that the fluorescence of these Trps in human gammaD-crystallin is very efficiently quenched in the na

126 gammaS-Crystallin is a highly stable structural protein

127 gammaS-Crystallin is a highly stable, abundant structura

128 gammaS-crystallin is a major human lens protein found in

129 Unlike other gamma-crystallins it has relatively low solubility, wher

130 ytic processing of the abundant lens protein gamma-crystallin, leading to its aggregation and subsequ

131 nd beta-crystallins and increased acidity of gamma-crystallins may cause insolubilization during agin

132 The present results suggest that gammaD-crystallin may polymerize through successive doma

133 ing of Trp fluorescence of human gammaD- and gammaS-Crystallin may protect them from ambient light in

134 ration and decreased expression of beta- and gamma-crystallins, MIP26, CP49, and filensin.

135 s with in vitro unfolding and aggregation of gamma-crystallins, mouse mutant substitutions were intro

136 Our findings of gamma-crystallin mRNA and protein expression in the reti

137 High levels of expression of a form of gamma-crystallin mRNA in mouse retina have been identifi

138 ology of aggregates formed by the P23T human gammaD-crystallin mutant associated with congenital cata

139 otype, we transgenically expressed different gammaD-crystallin mutants in the zebrafish lens and obse

140 Of particular interest were gammaB- and gammaD-crystallin mutants linked to dominant cataracts i

141 to study the mechanism of aggregation of two gammaD-crystallin mutants, W42R and W42Q: the former a c

142 ited murine cataracts involving this type of gamma-crystallin mutation, large inclusions containing t

143 ract is associated with aggregation of human gammaD-crystallins, one of the longest-lived proteins.

144 interactions were observed with the beta- or gamma-crystallins, or the cytoskeletal proteins actin, a

145 similar to the X-ray structure of wild-type gammaD-crystallin, pivotal local conformational and dyna

146 s retained the ability to differentiate into gamma-crystallin-positive lentoids by high-dosage bFGF t

147 ), and the second type of alpha-, beta-, and gamma-crystallins (possibly fragments) and two beaded fi

148 s, except for an additional acidic form of a gamma-crystallin, possibly due to a polymorphism.

149 Identification of the gamma-crystallin precursors to aggregates is crucial for

150 Surprisingly, wild-type gammad-crystallin promoted W42Q polymerization in a cata

151 but only occasionally leads ectopic sites of gamma-crystallin protein expression in select anterior h

152 MIP26 and gamma-crystallin protein expression was detected in conf

153 The loss of gamma-crystallin protein in cloche was not due to lowere

154 The detection of total gamma-crystallin protein in the retina was performed usi

155 not due to lowered mRNA levels but rather to gamma-crystallin protein insolubility.

156 Gamma-crystallin proteins were also detectable in murine

157 on in aqueous ternary solutions of calf lens gamma-crystallin proteins.

158 the aggregation pathways of the three human gamma-crystallin proteins.

159 These results indicate that gammaS-crystallin provides an excellent model system for

160 e gene product alters protein folding of the gamma-crystallin(s) and results in lens opacity.

161 e Arg-58 to His and Arg-36 to Ser mutants of gammaD crystallin show that the mutations dramatically l

162 Model studies showed that once cleaved from gammaS-crystallin, SPAVQSFRRIVE adopts a markedly differ

163 All of the gamma-crystallins studied formed large aggregates (or "m

164 lt to identify the conformation of the human gamma-crystallin substrate species recognized by human a

165 ormed long-lived stable complexes with their gammaD-crystallin substrates.

166 in structure resembling a trimer of beta- or gamma-crystallin subunits.

167 ces the phase separation temperatures of the gamma-crystallins, suggests that gamma(s)-crystallin pla

168 e S-methylation in soluble than in insoluble gammaS-crystallins supports the contention that S-methyl

169 ggregation of and refold the model substrate gammad-crystallin, suppress aggregation of mutant huntin

170 vent in cataract formation involving altered gamma-crystallins that are un likely to adopt their nati

171 For example, the expression of beta- and gamma-crystallins, the marker proteins for fiber differe

172 upregulate the fiber cell markers beta- and gamma-crystallins, the transcription factors cMaf and Pr

173 In human gammaD-crystallin, the P23T mutant is associated with co

174 iated with a mutation in Crygs, the gene for gammaS-crystallin, the first mutation to be associated w

175 Besides providing new information about gammaD-crystallin, this study demonstrates the complemen

176 An increased level of binding of beta- and gamma-crystallin to the alpha-crystallin fraction was ob

177 Gamma-crystallins undergo aggregation in which a small f

178 ted for a two-domain 177-amino-acid protein, gammaS crystallin, using an experimental SAXS data set f

179 Intramolecularly cross-linked gamma-crystallin was first separated from reaction side

180 e in the 129alpha3Cx46-/- mouse, cleavage of gamma-crystallin was not detected in the dKO mouse.

181 However, insolubilization of gamma-crystallins was associated with a decrease in isoe

182 in an Arg- 14 --> Cys (R14C) substitution in gammaD-crystallin was subsequently identified.

183 By teenage years, insoluble intact gammaS-crystallin was detected, indicative of protein de

184 nal differentiation markers such as beta- or gamma-crystallin were not induced.

185 Transcripts encoding most beta- and gamma-crystallins were detectable and, in some cases, ab

186 The alpha-, beta- and gamma-crystallins were found in distinct subcellular loc

187 ion, large inclusions containing the altered gamma-crystallins were found in the nuclei of the primar

188 By immunocytochemistry, gamma-crystallins were localized particularly to the inn

189 gammaD-Crystallins were not degraded by the UPP.

190 betaB1- and gammaS-crystallins were abundant in adult human, mouse,

191 ogy, age-related changes to a major protein, gammaS-crystallin, were studied.

192 rising increase in expression of cytoplasmic gamma-crystallin, whereas no changes in beta-crystallin

193 This may indicate a loss of function of gammaS-crystallin which would be consistent with ideas t

194 he natively monomeric human eye lens protein gammad-crystallin, whose aggregation leads to cataract d

195 Human gammaC belongs to a group of gamma-crystallins with a pair of cysteine residues at po

196 Binding of substrate gamma-crystallins with two or three of the four buried t

197 ing intermediate species of monomeric native gammaD-crystallin with a largely folded C-terminal domai

198 Derivatization of gammaS-crystallin with iodoacetamide showed reaction at