ライフサイエンスコーパス: cybrid

1 create a panel of 232 cytonuclear genotypes (cybrids).

2 ecrease in mitochondrial translation in this cybrid.

3 ene content could be the cause of CMS in the cybrid.

4 production of reactive oxygen species in the cybrids.

5 Ser(UCN)) level, compared with three control cybrids.

6 ity for the production of transmitochondrial cybrids.

7 y this procedure are true transmitochondrial cybrids.

8 lied on the production of transmitochondrial cybrids.

9 ions in mtDNA-encoded polypeptides in mutant cybrids.

10 35-year-old woman resulted in 71 synaptosome cybrids.

11 3460A mutation transferred with the mtDNA in cybrids.

12 rm line by means of embryonic stem (ES) cell cybrids.

13 sp) in mutant cybrids, compared with control cybrids.

14 tion of oxidative reactive species in mutant cybrids.

15 ROS generation was elevated in the AD cybrids.

16 anti-apoptotic protein Bcl-xL in the mutant cybrids.

17 n led to the respiratory phenotype in mutant cybrids.

18 rts on mitochondrial genome recombination in cybrids.

19 ion of reactive oxygen species in the mutant cybrids.

20 e consumption and lactate production than WT cybrids.

21 d showed higher metastatic potential than WT cybrids.

22 His in mutant cybrids, compared with control cybrids.

23 ltered bioenergetic profiles compared with H cybrids.

24 , and sorbitol levels were increased in LHON cybrids.

25 Increased AD cybrid Abeta(1-40) secretion was normalized by inhibitio

26 AD cybrids also displayed an increased basal cytosolic calc

27 All 7 patients were used for mixed cybrid analysis and demonstrated a selective 25% deficie

28 nature of mitochondrial recombination in the cybrid and to uncover the molecular mechanism of male fe

29 lts show that in hypoxic conditions, Euro/DM cybrids and [Afr + Asi]/DM cybrids show similar decrease

30 l alterations were shared among osteosarcoma cybrids and lymphoblasts bearing LHON mutations.

31 ductase transcript was overexpressed in LHON cybrids and lymphoblasts.

32 40% CI-inhibited human-ape xenomitochondrial cybrids) and a drug-induced model (0-100% CI-inhibited c

33 ssect the role of mtDNA in cancer, including cybrids, and more recently mitochondrial-nuclear exchang

34 maintained in at least a proportion of A549 cybrids, and suggest that the complex I defect in dyston

35 This mouse ES cell-cybrid approach now provides the means to generate a wid

36 at differences in epigenetic status found in cybrids are likely due to single nucleotide polymorphism

37 Transmitochondrial cybrid systems (cybrids) are an excellent tool to study specific effects

38 sses tau oligomer accumulation in MCI and AD cybrids as well as cortical neurons from tau mice.

39 type (T8993T) cybrids, whereas the wild-type cybrids barely grew in the mice.

40 We demonstrated that these cybrids bearing only m.3394T>C mutation caused mild mito

41 Moreover, the cybrids bearing the m.14484T > C mutation exhibited the

42 These new transmitochondrial cybrids became defective once again in oxidative phospho

43 detailed analyses of the mtDNA of a repeated cybrid between the solanaceae Nicotiana tabacum and Hyos

44 y demonstrates that HepG2 transmitochondrial cybrids can be created to contain the mitochondrial geno

45 by, respiratory-deficient transmitochondrial cybrids can be isolated.

46 et when human TIM17A is overexpressed in NT2 cybrids carrying A3243G mtDNA, the proportion of cybrid

47 tment with the mTORC1 inhibitor rapamycin in cybrids carrying either large-scale partial deletions of

48 d regulation capacity has been observed with cybrids carrying mtDNA from skeletal muscle of old mice.

49 ited greater mitochondrial dysfunctions than cybrids carrying only m.11778G>A mutation.

50 we report the successful generation of mouse cybrids carrying skeletal muscle mtDNA.

51 Similar to their human counterparts, cybrids carrying the homoplasmic mouse LHON mutation dem

52 We have used a patient cybrid cell line with a single point mutation in the ove

53 We isolated each of the mtDNAs in a separate cybrid cell line.

54 was observed in symptomatic or asymptomatic cybrid cell lines carrying the C1494T mutation as compar

55 ochondrial 12S rRNA C1494T mutation using 27 cybrid cell lines constructed by transferring mitochondr

56 The resulting cybrid cell lines contained the same nuclear genotype an

57 evance of this particular mutation in vitro, cybrid cell lines containing different mt-Tr (tRNA(Arg))

58 MPD/ascorbate-promoted respiration in mutant cybrid cell lines derived from either symptomatic or asy

59 e m(1)G37 modification of mt-tRNA(Asp) Using cybrid cell lines generated by transferring mitochondria

60 the molecular players involved, we generated cybrid cell lines harbouring either wild-type (WT) or mu

61 o0 cells with human platelets yielded clonal cybrid cell lines that were populated exclusively with d

62 Mixed cybrid cell lines were analyzed for 9 controls and 9 dys

63 After 6 weeks of culture, cybrid cell lines were assayed for complex I activity an

64 Since cybrid cell lines with 73% m.3243A > G heteroplasmy (DW7

65 gene expression, which are recapitulated in cybrid cell lines with different percentages of m.3243G

66 This study created 10 cybrid cell lines, each containing distinct mitochondria

67 ogical characteristics of transmitochondrial cybrid cell lines, obtained by fusing of platelets from

68 ) mouse or human cell lines result in viable cybrid cell lines.

69 n=10), thus generating 10 transmitochondrial cybrid cell lines.

70 We performed functional assays using the cybrid cell models, generated by fusing mtDNA-less rho(o

71 Using cell culture model and cybrid cell technology, we provide evidence that mitocho

72 amount of total mtDNA was 3800+/-1600 copies/cybrid cell, and the average percentage of heteroplasmy

73 These cybrids cell lines bearing m.14502T > C mutation exhibit

74 tation levels in a human cytoplasmic hybrid (cybrid) cell line expressing a heteroplasmic mtDNA G1177

75 , and transmitochondrial cytoplasmic hybrid (cybrid) cell lines are the most frequently used model fo

76 atus in a panel of human cytoplasmic hybrid (cybrid) cell lines carrying a variety of pathogenic mtDN

77 weeks in culture, these cytoplasmic hybrid (cybrid) cell lines were assayed for electron transport c

78 itochondrial leucyl-tRNA synthetase into the cybrid cells carrying the A3243G mutation improved the e

79 ndrial defective metabolism by treating LHON cybrid cells carrying the m.11778G>A mutation with a com

80 sted the sensitivity of osteosarcoma-derived cybrid cells carrying the most common and severe mutatio

81 Individual cybrid cells contained 100-2600 copies of wild-type mtDN

82 l analysis was then performed in primary and cybrid cells containing candidate mutations identified d

83 whether involving toxins, mutated genes, or cybrid cells containing patient mitochondria.

84 and IV were confirmed in transmitochondrial cybrid cells containing the m.12955A > G mutation, sugge

85 Expression of AD mitochondrial genes in cybrid cells depresses cytochrome c oxidase activity and

86 hibition in Caenorhabditis elegans and human cybrid cells improved the heteroplasmy ratio and restore

87 After relieving cybrid cells of Parkin overexpression, a more favorable

88 esults obtained with human xenomitochondrial cybrid cells were compatible with those observed in rote

89 sphate (ATP) synthesis rates of osteosarcoma cybrid cells were measured before and after CPC and BAK

90 These cybrid cells were subjected to mitochondrial genome sequ

91 ow that functional differences exist between cybrid cells which differ in mitochondrial genomic backg

92 in Alzheimer's disease-derived mitochondrial cybrid cells, an ex vivo human sporadic Alzheimer's dise

93 substrate, restored the hypoxic response in cybrid cells, suggesting that electron transport chain a

94 deleterious COXI mutations in heteroplasmic cybrid cells, thereby enriching cells for wild-type mtDN

95 sensitive, metabolically impaired rho+ mouse cybrid cells.

96 rt activities were restored to normal in the cybrid cells.

97 OX5a protein levels in complex I (CI) mutant cybrid cells.

98 and MCI-derived mitochondria and AD and MCI cybrid cells.

99 Cytoplasmic hybrid ("cybrid") cells made from mitochondrial DNA of nonfamilia

100 ynthetase (LARS2) in the cytoplasmic hybrid (cybrid) cells carrying the A3243G mutation corrects the

101 Three percent of the cybrid clones had mtDNAs with 10 Cs, 76% had nine, 18% h

102 ids carrying A3243G mtDNA, the proportion of cybrid clones maintaining mtDNA increases significantly.

103 Sequencing the mtDNA control region of these cybrid clones revealed differences in the number of Cs i

104 as significantly increased in [Afr + Asi]/DM cybrids compared to Euro/DM cybrids in hypoxic condition

105 steady-state level of mt-tRNA(Asp) in mutant cybrids, compared with control cybrids.

106 the steady-state level of tRNAHis in mutant cybrids, compared with control cybrids.

107 Relative to mock-transfected G11778A cybrids, complemented G11778A cybrids showed a threefold

108 used with A549 p0 (mtDNA-less) cells to form cybrids comprising the A549 nucleus and dystonia mtDNA.

109 T3308C and tRNA(Ala) T5655C mutations using cybrids constructed by transferring mitochondria from ly

110 Mutant cybrids constructed by transferring mitochondria from ly

111 Using cybrids constructed by transferring mitochondria from ly

112 ion with pyruvate, a complex I substrate, in cybrids containing 60% to 90% 3243G:C mitochondrial DNA.

113 However, in A3460G LHON fusion cybrids containing a different nuclear background, A549

114 We have isolated transmitochondrial cybrids containing a mitochondrial DNA cytochrome b 4-ba

115 grown in hypoxic conditions versus room-air, cybrids containing mitochondria from African and Asian d

116 ifferentiated from mouse embryonic stem-cell cybrids containing mitochondrial DNA polymorphic variant

117 s, to a level indistinguishable from that in cybrids containing normal mitochondrial DNA.

118 ated in individual cytoplasmic hybrid cells (cybrids), containing a large mtDNA deletion, and bulk ce

119 for haplogroups so we created human ARPE-19 cybrids (cytoplasmic hybrids), which have identical nucl

120 from tau mice and human trans-mitochondrial 'cybrid' (cytoplasmic hybrid) neuronal cells, whose mitoc

121 AD cybrids demonstrated a 52% decrease in electron transpor

122 Three cybrids derived from an affected matrilineal relative ca

123 labeling, respectively, compared with twelve cybrids derived from four Chinese control individuals.

124 ived from two asymptomatic members, and nine cybrids derived from three symptomatic members of the Ch

125 Six cybrids derived from two asymptomatic members, and nine

126 tRNAmut cybrids displayed increased motility and migration capac

127 iles compared to European/diabetic (Euro/DM) cybrids (e.g., fatty acid metabolism: EnrichR rank 10 in

128 In primary myoblasts and transmitochondrial cybrids established from the proband (index case) and of

129 tRNAmut cybrids exhibited lower oxygen consumption and higher gl

130 The Large White and Xiang cybrids exhibited similar mtDNA copy numbers and differe

131 he 3460A and 11778A mutations transferred in cybrid experiments linking these defects to the mtDNA.

132 lonal analysis of A549 p0/PD platelet fusion cybrids from 1 of the patients expressed combined comple

133 We investigated the bioenergetics of cybrids from five patients carrying different ATP6 mutat

134 lonal neuronal cells hybridized with mtDNA ('cybrids') from PD or AD patients.

135 we developed an enhanced cytoplasmic hybrid (cybrid) generation protocol and established isogenic hum

136 oss of function rescues death of CI-impaired cybrids grown under conditions requiring OXPHOS activity

137 at the residual tRNA(Asn) fraction in mutant cybrids had an altered conformation, suggesting that the

138 ess cell line to generate transmitochondrial cybrids harboring different proportions of mutated and w

139 e now show that, although transmitochondrial cybrids harboring homoplasmic levels of the mutation do

140 deficiency as well as in transmitochondrial cybrids harboring mitochondrial encephalomyopathy lactic

141 he parental mouse cells or xenomitochondrial cybrids harboring Mus spretus mtDNA.

142 Mouse xenomitochondrial cybrids harboring rat mtDNA had a slower growth rate in

143 rotein was imported into the mitochondria of cybrids harboring the G11778A mutation, where it increas

144 Cybrids harboring the np 14459 mutation exhibited a 39%

145 fects in lymphoblasts and transmitochondrial cybrids harboring the three most common LHON mutations:

146 molecular and biochemical characteristics of cybrids harboring varying levels of mutated mitochondria

147 ear genes by constructing transmitochondrial cybrids harbouring mitochondria with either haplogroup H

148 J cybrids have altered bioenergetic profiles compared with

149 Analyses revealed that untreated-H and -J cybrids have different expression levels for nuclear gen

150 J and H cybrids have significantly altered expression of eight n

151 was amplified and cloned from a synaptosome cybrid homoplasmic for a mtDNA with nine Cs.

152 ansfected constructs in cytoplasmic hybrids (cybrids) homoplasmic with respect to the 8993T-->G mutat

153 cybrids presented lower growth rate than WT cybrids, however, when injected in nude mice, tRNAmut cy

154 mpanzee, and human-gorilla xenomitochondrial cybrids (HXC).

155 plex I-inhibited human-ape xenomitochondrial cybrids, hypoxic induction of HIF-1alpha was severely re

156 n [Afr + Asi]/DM cybrids compared to Euro/DM cybrids in hypoxic conditions.

157 The increasing feasibility of producing cybrids in various species calls for further research in

158 p 14459 mutation by using transmitochondrial cybrids in which patient Epstein-Barr virus-transformed

159 t report where the mitochondrial genome of a cybrid is compared with its actual parents.

160 This result in transmitochondrial cybrids is in contrast to the differences in the same pa

161 Although the procedure to produce such cybrids is well established, it is laborious and cumbers

162 A cybrid line with a very high level of 3243G:C mitochondr

163 ex I-specific activity relative to wild-type cybrid lines but normal activity for the other complexes

164 Eight of the 15 cybrid lines contained mtDNA obtained from maternally de

165 ength variation was found in patient-derived cybrid lines containing 0-97.5% 3243 G:C.

166 These findings were present in cybrid lines containing mtDNA from maternal descendants

167 containing mtDNA from paternal descendants, cybrid lines containing mtDNA from maternal descendants

168 Compared with the cybrid lines containing mtDNA from paternal descendants,

169 We have isolated several transmitochondrial cybrid lines harboring this mutation, one of which (clon

170 controls and 9 dystonia patients, and clonal cybrid lines were generated for 2 control and 2 dystonia

171 ocol and established isogenic human melanoma cybrid lines with wild-type mtDNA or pathogenic mtDNA mu

172 The resulting transmitochondrial cybrid lines, containing either exclusively wild-type or

173 omplemented in both the mixed and the clonal cybrid lines.

174 In human cytoplasmic hybrid (cybrid) lines, fMet modulated both mitochondrial and cyt

175 ombination mechanisms were recognized in the cybrid mitochondria.

176 The majority of the studies using the cybrid model focused on the significance of specific mit

177 show, by using the human transmitochondrial cybrid model, that the Cterm is also able to improve the

178 Using cybrid models for LHON, we show that autophagy is signif

179 The brain origin of mouse synaptosome cybrid mtDNAs was confirmed using sequence polymorphisms

180 c alterations was studied in cell-engineered cybrids Nicotiana tabacum (+ Hyoscyamus niger) combining

181 ed, cytoplasmic male sterile Brassica juncea cybrid Og1 derived from Ogura cytoplasm shows frequent r

182 the cytoplasmic male sterile Brassica juncea cybrid Og1.

183 in complex I activity compared with control cybrids or SH-SY5Y cells.

184 in long-term mtDNA stabilization, since NT2 cybrids overexpressing TIM17A maintain mtDNA at levels s

185 The resulting ES cell cybrids permitted transmission of the NZB mtDNAs through

186 Cybrid plant mitochondria undergo homologous recombinati

187 The cybrid plants were grown in a range of different light a

188 Cybrids prepared from the fusion of enucleated fibroblas

189 When cultured in vitro, tRNAmut cybrids presented lower growth rate than WT cybrids, how

190 however, when injected in nude mice, tRNAmut cybrids produced larger tumours and showed higher metast

191 In conclusion, transmitochondrial cybrids provide the first direct evidence on pig biochem

192 d ETC activity and partially restored the AD cybrid recovery rate.

193 The biochemical analysis of the cybrids revealed that the mutant haplotype is associated

194 nditions, Euro/DM cybrids and [Afr + Asi]/DM cybrids show similar decreases in ROS production.

195 The G11778A cybrids showed a 60% reduction in the rate of ATP synthe

196 fected G11778A cybrids, complemented G11778A cybrids showed a threefold increase in ATP synthesis, to

197 Moreover, these cybrids showed decreased synthesis of a number of polype

198 All cybrids showed decreased ZO1-minus protein levels, but t

199 PGC-1alpha and PGC-1beta in the osteosarcoma cybrids stimulated mitochondrial respiration suggesting

200 The cybrid strain also displayed significantly increased CIV

201 on nuclear background in cytoplasmic hybrid (cybrid) strains, we were able to quantify the among-line

202 Transmitochondrial cybrid systems (cybrids) are an excellent tool to study

203 Using cybrid technology, we found that in a high-glucose mediu

204 steosarcoma nuclear background (osteosarcoma cybrids), the rate of respiration markedly declined sugg

205 o unaltered in successful "xenomitochondrial cybrids." The abrupt failure of mtDNA from primate speci

206 litates the production of transmitochondrial cybrids, thereby increasing the number of mtDNA mutation

207 the entire mtDNA was carried out for all the cybrids to identify haplogroup and non-haplogroup defini

208 neration of HepG2-derived transmitochondrial cybrids, to investigate the impact of mtDNA variation on

209 to the PC3 prostate cancer cell line through cybrid transfer and tested for tumor growth in nude mice

210 and segregation in human cells using serial cybrid transfer of partially duplicated mitochondrial DN

211 of mtDNA maintenance frequently follow from cybrid transfer.

212 In heteroplasmic cybrid tumors, single-cell analyses revealed selection a

213 ained by growing the 8AGr transmitochondrial cybrids under selection.

214 notype of the LHON genotype, we have created cybrids using a neuronal precursor cell line, Ntera 2/D1

215 The mtDNA of the cybrid was intermediate between the size of the parental

216 to the total transmitochondrial hybrids and cybrids was approximately 1% and no hybrids were isolate

217 The brain origin of the human synaptosome cybrids was confirmed using a rare mtDNA Mbo I polymorph

218 g that increased Fas-dependent death in LHON cybrids was induced by the LHON pathogenic mutations.

219 of complex I activity were normal in G11778A cybrids we focused on changes in ATP synthesis using com

220 In PD cybrids we found a stable 20% decrement in complex I act

221 These cybrids were enucleated and the cytoplasts were electrof

222 Cybrids were established by fusing the mitochondria DNA

223 The resulting mutant (T8993G) cybrids were found to generate tumors that were 7 times

224 To address this issue, cybrids were generated by fusing osteosarcoma cells devo

225 A haplotypes, two porcine transmitochondrial cybrids were generated by fusion of a Lantang pig cell l

226 We observed that LHON cybrids were sensitized to Fas-dependent death.

227 Synaptosome cybrids were used to confirm the presence of heteroplasm

228 Cytoplasmic hybrids (cybrids) were created for 15 family members over two gen

229 equences, mitochondrially transformed cells (cybrids) were created from AD patients or disease-free c

230 e 7 times larger than the wild-type (T8993T) cybrids, whereas the wild-type cybrids barely grew in th

231 or mutant mitochondrial DNA (mtDNA) [tRNAmut cybrids, which harbour the pathogenic A3243T mutation in

232 ed comparison analyses of human retinal cell cybrids, which possess identical nuclei, but mtDNA from

233 observed with the mitochondria donor cells, cybrids with benign mitochondria showed high mitochondri

234 that several oncogenic pathways observed in cybrids with cancer mitochondria are inhibited in cybrid

235 and respiratory chain activities compared to cybrids with cancerous mitochondria.

236 Cybrids with homoplasmic levels of pathogenic mtDNA reli

237 ds with cancer mitochondria are inhibited in cybrids with non-cancerous mitochondria.

238 Analysis of transmitochondrial cybrids with the 13708A-H7 mtDNA revealed a lower mitoch

239 iochemical assays were performed on selected cybrids with various proportions of the two types of mit

240 A transmitochondrial cybrid worm strain, chpIR (M, CB4856>N2), was bred as ho