ライフサイエンスコーパス: cybrid

1 ecrease in mitochondrial translation in this cybrid.

2 ions in mtDNA-encoded polypeptides in mutant cybrids.

3 n led to the respiratory phenotype in mutant cybrids.

4 ity for the production of transmitochondrial cybrids.

5 y this procedure are true transmitochondrial cybrids.

6 lied on the production of transmitochondrial cybrids.

7 35-year-old woman resulted in 71 synaptosome cybrids.

8 3460A mutation transferred with the mtDNA in cybrids.

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

10 ROS generation was elevated in the AD cybrids.

11 e consumption and lactate production than WT cybrids.

12 d showed higher metastatic potential than WT cybrids.

13 His in mutant cybrids, compared with control cybrids.

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

15 ltered bioenergetic profiles compared with H cybrids.

16 tion of oxidative reactive species in mutant cybrids.

17 , and sorbitol levels were increased in LHON cybrids.

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

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

20 AD cybrids also displayed an increased basal cytosolic calc

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

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

23 ductase transcript was overexpressed in LHON cybrids and lymphoblasts.

24 40% CI-inhibited human-ape xenomitochondrial cybrids) and a drug-induced model (0-100% CI-inhibited c

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

70 These cybrid cells were subjected to mitochondrial genome sequ

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

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

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

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

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

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

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

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

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

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

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

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

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

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

85 Using cybrids constructed by transferring mitochondria from ly

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

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

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

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

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

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

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

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

94 AD cybrids demonstrated a 52% decrease in electron transpor

95 Three cybrids derived from an affected matrilineal relative ca

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

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

98 Six cybrids derived from two asymptomatic members, and nine

99 tRNAmut cybrids displayed increased motility and migration capac

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

101 tRNAmut cybrids exhibited lower oxygen consumption and higher gl

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

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

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

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

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

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

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

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

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

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

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

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

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

115 Cybrids harboring the np 14459 mutation exhibited a 39%

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

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

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

119 J cybrids have altered bioenergetic profiles compared with

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

148 Cybrids prepared from the fusion of enucleated fibroblas

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

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

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

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

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

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

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

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

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

158 The cybrid strain also displayed significantly increased CIV

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

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

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

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

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

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

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

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

167 of mtDNA maintenance frequently follow from cybrid transfer.

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

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

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

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

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

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

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

175 These cybrids were enucleated and the cytoplasts were electrof

176 Cybrids were established by fusing the mitochondria DNA

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

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

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

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

181 Synaptosome cybrids were used to confirm the presence of heteroplasm

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

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

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

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

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

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

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

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

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

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

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