ライフサイエンスコーパス: SP-C

1 SP-C associates with surfactant lipids to reduce surface

2 SP-C is a small alpha-helical hydrophobic protein with a

3 SP-C is prone to convert into beta-sheet aggregates, for

4 SP-C mature peptide is stored in lamellar bodies (a lyso

5 SP-C mRNA was not detected in the lungs of SP-C (-/-) mi

6 SP-C mRNA, however, was expressed only in type II cells,

7 SP-C plays an important role in innate host defense of t

8 SP-C precursor protein displayed aberrant subcellular lo

9 SP-C represents a structurally and functionally challeng

10 SP-C secretion in null mice was restored by the addition

11 SP-C shows complex oligomerization behavior and a transi

12 SP-C-deficient (SP-C -/-) mice developed a severe pulmon

13 lveolar wash were 4.9 times higher in SP-A + SP-C-surfactant-treated animals.

14 of RLE-6TN cells expressing these SP-A1 and SP-C variants suggesting that anti-TGF-beta therapeutics

15 re and common surfactant protein (SP)-A1 and SP-C variants, either discovered in our familial pulmona

16 Our results suggest that SP-B and SP-C accelerate adsorption through a mechanism other tha

17 r understanding of the functions of SP-B and SP-C and the structural basis for their actions.

18 LS context, the combined action of SP-B and SP-C appears to facilitate cholesterol dynamics, whereas

19 Both SP-B and SP-C eliminate squeeze-out of POPG from mixed DPPG/POPG

20 and the potential roles of abnormal SP-B and SP-C expression and genetic variation in these genes in

21 es associated with mutations in the SP-B and SP-C genes, and the potential roles of abnormal SP-B and

22 The hydrophobic surfactant proteins SP-B and SP-C greatly accelerate the adsorption of vesicles conta

23 generation of non-natural mimics of SP-B and SP-C has previously been restricted to step-by-step, seq

24 The overlap of function between SP-B and SP-C helps explain why replacement surfactants lacking i

25 py of pulmonary surfactant proteins SP-B and SP-C in lipid-protein monolayers at the air-water interf

26 y lung surfactant specific proteins SP-B and SP-C in monolayers of dipalmitoylphosphatidylglycerol (D

27 plore the possibility that proteins SP-B and SP-C induce the permeabilization of phospholipid membran

28 To determine if SP-B and SP-C might promote adsorption by inducing negative curva

29 e effect of the surfactant proteins SP-B and SP-C on cholesterol distribution in membranes.

30 on the surfactant-specific proteins SP-B and SP-C on the morphology and function of surfactant monola

31 The hydrophobic proteins SP-B and SP-C promote rapid adsorption of pulmonary surfactant to

32 The hydrophobic surfactant proteins SP-B and SP-C promote rapid adsorption of pulmonary surfactant to

33 All those results suggest that SP-B and SP-C proteins promote the formation of proteolipid chann

34 c lung surfactant specific proteins SP-B and SP-C that induce structural changes in the monolayer tha

35 f lung surfactant proteins B and C (SP-B and SP-C), two helical and amphiphilic proteins that are cri

36 For both SP-B and SP-C, a statistical windowed autocorrelation method iden

37 c proteins of lung surfactant (LS), SP-B and SP-C, are critical constituents of an effective surfacta

38 he hydrophobic surfactant proteins, SP-B and SP-C, greatly accelerate the adsorption of the surfactan

39 wo hydrophobic surfactant proteins, SP-B and SP-C, have been incorporated into therapeutic agents for

40 he hydrophobic surfactant proteins, SP-B and SP-C, have important roles in surfactant function.

41 including the hydrophobic proteins SP-B and SP-C, in charge of stabilizing the respiratory surface o

42 ce of the two hydrophobic proteins, SP-B and SP-C, on the thermodynamic barriers that limit adsorptio

43 wo hydrophobic surfactant proteins, SP-B and SP-C, which are thought to play pivotal roles in the ads

44 y), mean +/- SEM contents of SP-A, SP-B, and SP-C (3.7 kD) were 7.1 +/- 1.4%, 1.8 +/- 0.2%, and 4.6 +

45 ajor postnatal increases for SP-A, SP-B, and SP-C occurred during the 1st, 2nd, and 3rd weeks, respec

46 2nd day of life, contents of SP-A, SP-B, and SP-C were 13.4%, 8.4%, and 0.1%, respectively, of the me

47 ng morphology were similar in SP-C (-/-) and SP-C (+/+) mice.

48 ng Erm diminished expression of both Erm and SP-C but had no effect on beta-actin or GAPDH (glycerald

49 Lung pathology in both WT and SP-C-hCFTR+/+ mice was marked by neutrophilic inflammati

50 Intranasally-administered, anti-SP-C-conjugated lipoplexes targeted mouse ATII cells wit

51 48h after treatment with anti-SP-C-conjugated lipoplexes containing the test microRNA

52 onstrate the importance of using appropriate SP-C-rtTA only controls in all experiments.

53 However, surfactant proteins SP-B, SP-C, and Clara cell secretory protein, normally produce

54 rfactant-associated proteins A (SP-A), SP-B, SP-C, and SP-D; Clara cell-associated protein CC-10; and

55 ctures are detected in the presence of SP-B, SP-C, or the native mixture of both proteins.

56 t lipids and proteins, including SP-A, SP-B, SP-C, SP-D, ABCA3 (a lamellar body associated protein) a

57 h solid and papillary, expressed SP-A, SP-B, SP-C, SP-D, and thyroid transcription factor-1, but not

58 nts provide evidence that the aromatic-based SP-C peptoid mimics, in conjunction with a synthetic lip

59 Peptoid-based SP-C mimics are easily produced and purified, and offer

60 and POPG motion seem to be more hindered by SP-C than dipalmitoylphosphatidylcholine.

61 Biosynthesis of surfactant protein C (SP-C) by alveolar type 2 cells requires proteolytic proc

62 Mutations in the surfactant protein C (SP-C) gene (SFTPC) are associated with familial desquama

63 The surfactant protein C (SP-C) gene encodes an extremely hydrophobic, 4-kDa pepti

64 Surfactant protein C (SP-C) has been suggested to be an essential element for

65 determine the role of surfactant protein C (SP-C) in host defense, SP-C-deficient (Sftpc-/-) mice we

66 ith those of TTF-1 and surfactant protein C (SP-C) in the respiratory epithelial cells of the mouse l

67 Surfactant protein C (SP-C) is a hydrophobic 35-amino acid peptide that co-iso

68 Surfactant protein C (SP-C) is a hydrophobic lipopeptide that is critical for

69 Bovine pulmonary surfactant protein C (SP-C) is a hydrophobic, alpha-helical membrane-associate

70 Surfactant protein C (SP-C) is a lung-specific protein that is synthesized as

71 Surfactant protein C (SP-C) is a novel amyloid protein found in the lung tissu

72 Surfactant Protein C (SP-C) is a secreted transmembrane protein that is exclus

73 Rat surfactant protein C (SP-C) is synthesized as a 194-amino acid propeptide (SP-

74 R in the lung from the surfactant protein C (SP-C) promoter (SP-C-hCFTR+/-).

75 specific promoter--the surfactant protein C (SP-C) promoter.

76 he lung-specific human surfactant protein C (SP-C) promoter.

77 e under control of the surfactant protein C (SP-C) promoter.

78 teolytic processing of surfactant protein C (SP-C) proprotein in multivesicular bodies of alveolar ty

79 adation of the encoded surfactant protein C (SP-C) proprotein.

80 the encoded proprotein surfactant protein C (SP-C) trigger endoplasmic reticulum (ER)-associated degr

81 , pulmonary surfactant-associated protein C (SP-C), L-plastin, annexin A1, and haptoglobin increased,

82 n of the gene encoding surfactant protein C (SP-C), was associated with either absent or decreased ex

83 Expression of surfactant protein C (SP-C), which is restricted to alveolar type II epithelia

84 holipids is surfactant-associated protein C (SP-C).

85 Surfactant protein C (SP-C; Sftpc) gene expression is restricted to pulmonary

86 from either the human surfactant protein-C (SP-C) or rat Clara cell secretory protein (ccsp) genes.

87 ct of the 3.7-kb human surfactant protein-C (SP-C) promoter and the rat PGIS cDNA.

88 cell-specific antigen surfactant protein-C (SP-C) then administered to C57BL/6 mice via the nares.

89 exon4, generating a truncated form of SP-C (SP-C(Deltaexon4)).

90 ependently driven by the human surfactant C (SP-C) promoter.

91 ted to CD63 (+) vesicles that also contained SP-C-(1-194).

92 In contrast, SP-C-GM mice exposed to hyperoxia demonstrated only mode

93 dependent upon the NH2-terminal cytoplasmic SP-C propeptide, which contains a conserved PPDY motif.

94 surfactant protein C (SP-C) in host defense, SP-C-deficient (Sftpc-/-) mice were infected with the pu

95 SP-C-deficient (SP-C -/-) mice developed a severe pulmonary disorder ass

96 xtracellular matrix production distinguished SP-C/TNF-alpha mice from fibrosis models.

97 protein (EGFP) linked to SP-C-(1-194) (EGFP/SP-C-(1-194)), to mutant proSP-C lacking the NH(2) targe

98 ant that forms juxtanuclear aggregates, EGFP/SP-C(C122/186G), was not corrected by cotransfection wit

99 P-C-(24-194)), or to mature SP-C alone (EGFP/SP-C-(24-58)) were produced.

100 y revealed that pcDNA3/SP-C-(1-194) and EGFP/SP-C-(1-194) were each expressed in CD63 (+), EEA1 (-) c

101 -C-(1-194), both EGFP/SP-C-(24-194) and EGFP/SP-C-(24-58) were directed to CD63 (+) vesicles that als

102 ric forms of both EGFP/SP-C-(1-194) and EGFP/SP-C-(24-58).

103 exane produced multimeric forms of both EGFP/SP-C-(1-194) and EGFP/SP-C-(24-58).

104 nsfected with pcDNA3/SP-C-(1-194), both EGFP/SP-C-(24-194) and EGFP/SP-C-(24-58) were directed to CD6

105 P-C lacking the NH(2) targeting domain (EGFP/SP-C-(24-194)), or to mature SP-C alone (EGFP/SP-C-(24-5

106 Expression of EGFP/SP-C-(24-194) or EGFP/SP-C-(24-58) resulted in transloca

107 Expression of EGFP/SP-C-(24-194) or EGFP/SP-C-(24-58) resulted in translocation but retention in

108 e peptoid-based mimics studied here emulated SP-C's secondary structure, forming stable helical struc

109 tratracheal injection of adenovirus encoding SP-C mature peptide resulted in secretion into the alveo

110 cDNA encoding SP-C(Deltaexon4) was constitutively expressed in type II

111 Mutations in the gene encoding SP-C (SFTPC) have recently been linked to chronic lung d

112 Mutations in the gene encoding SP-C (surfactant protein C; SFTPC) have been linked to i

113 disease, and mutations in the gene encoding SP-C are associated with chronic interstitial lung disea

114 However, the infection of cells expressing SP-C(Deltaexon4) with respiratory syncytial virus result

115 Here we report that DOX-fed SP-C-rtTA mice during the period in which Type II cells

116 quantitative analysis reveled that DOX-fed, SP-C-rtTA C57BL/6 pups had reduced surfactant mRNA accum

117 For SP-C, 2D IR and betanu correlation analyses of these reg

118 pro-SP-C, which functions as a chaperone for SP-C during biosynthesis.

119 Our results suggest a potential role for SP-C in generating small surfactant structures that may

120 ermediate directly abstracts a hydrogen from SP C-6 to generate a substrate radical, and subsequent t

121 d less hydrogen peroxide than did cells from SP-C-surfactant-treated animals.

122 cted by Western blot of alveolar lavage from SP-C (-/-) mice.

123 The morphology of the lungs from SP-C/HFH-4 embryos (day 18 postconception) was distinctl

124 ) was increased in alveolar macrophages from SP-C (-/-) mice.

125 o discern the role of SP-C in lung function, SP-C-deficient (-/-) mice were produced.

126 pression of the lung epithelial marker genes SP-C, SP-B and SP-A.

127 bacteria was also similar in WT, hemizygous SP-C-hCFTR+/-, and bitransgenic gut-corrected FABP-hCFTR

128 killing was observed in lungs of homozygote SP-C-hCFTR+/+ mice.

129 These results shed some light on how SP-C-induced lipid perturbations can alter membrane stru

130 However, SP-C expression was slightly diminished in larger (older

131 However, SP-C's posttranslational modification with N-terminal pa

132 re replaced with D/A were developed by human SP-C promoter-driven overexpression of the D/A gene in S

133 en treated with 100 mg/ kg recombinant human SP-C surfactant or with the same surfactant supplemented

134 on in surfactant function, we used the human SP-C promoter to drive expression of rat SP-A (rSPA) or

135 These findings implicate SP-C as a modifier of alveolar homeostasis.

136 he post-translational modifications found in SP-C, affords further improvements by reducing the perce

137 in contrast, lung structure and function in SP-C null mice is normal.

138 However, in SP-C/TNF-alpha-transgenic mice, both the numbers of eosi

139 Microarray screens for genes involved in SP-C ER-associated degradation identified MKS3/TMEM67, a

140 results demonstrate that Erm is involved in SP-C regulation, which results from an interaction with

141 days in hyperoxia was significantly lower in SP-C-GM mice than in wild-type mice, indicating preserva

142 Lamellar bodies were present in SP-C (-/-) type II cells, and tubular myelin was present

143 Alveolar fluid clearance was preserved in SP-C-GM mice in hyperoxia, but decreased significantly i

144 nthesis, and lung morphology were similar in SP-C (-/-) and SP-C (+/+) mice.

145 aled less disruption of the alveolar wall in SP-C-GM mice compared to wild-type mice.

146 tion of the other site resulted in increased SP-C transcription.

147 o the medium, however, were unable to induce SP-C expression, which required the additional presence

148 NFIen in a subset of type II cells inhibited SP-C gene expression without affecting expression of TTF

149 Interestingly, SP-C exhibits a membrane-fragmentation behavior, leading

150 rimental constructs was driven by the 3.7-kb SP-C promoter.

151 reversed by the presence of the lipopeptide SP-C.

152 it is uncertain whether deficiency of mature SP-C contributes to disease pathogenesis.

153 ation of amyloid deposits composed of mature SP-C in lung tissue samples from ILD patients with mutat

154 ropeptide and extramembrane domain of mature SP-C peptide, supported secretion of the transmembrane d

155 suggested the extramembrane domain of mature SP-C was cytosolic and sufficient for sorting to the reg

156 It is highly likely that mature SP-C adopts such a dimeric structure in the lamellar bil

157 n of proSP-C monomers mediated by the mature SP-C domain.

158 al anchor domain contained within the mature SP-C sequence and by a targeting domain in the NH(2)-fla

159 ng domain (EGFP/SP-C-(24-194)), or to mature SP-C alone (EGFP/SP-C-(24-58)) were produced.

160 the lungs of SP-C (-/-) mice, nor was mature SP-C protein detected by Western blot of alveolar lavage

161 at Erm significantly enhanced TTF-1-mediated SP-C transcription.

162 -substituted glycines, or peptoids, to mimic SP-C.

163 Misfolded SP-C, ERdj4, and ERdj5 coprecipitated with p97/VCP indic

164 on to chronic ER stress imposed by misfolded SP-C was associated with increased susceptibility to vir

165 Rdj4 and ERdj5 promote turnover of misfolded SP-C and this activity is dependent on their ability to

166 ntion and inhibited degradation of misfolded SP-C, but it had little effect on the wild-type protein.

167 d fluid at high surface tension and modifies SP-C function.

168 Mutational analysis of the 0.32-kb mouse SP-C promoter and transient transfection of MLE-15 cells

169 gistically activated the expression of mouse SP-C-luciferase reporter constructs.

170 The 4.8-kilobase murine SP-C promoter was used to generate 3 transgenic lines wh

171 ciated with patients heterozygous for mutant SP-C alleles.

172 teracted with a complex that included mutant SP-C and associated chaperones, whereas the region predi

173 ntially restored rapid degradation of mutant SP-C proprotein, whereas transfection of HPD mutants fai

174 h p97 and resulted in accumulation of mutant SP-C proprotein; knockdown of MKS3 also inhibited degrad

175 of MKS3 also inhibited degradation of mutant SP-C.

176 ot associate with either wild-type or mutant SP-C.

177 Expression of the index mutation, SP-C(Deltaexon4), in transiently transfected cells and t

178 the alveolar space of wild type mice but not SP-C (-/-) mice.

179 idue N-terminal peptide [SP-C13(palm)(2)] of SP-C, in mixtures with 1,2-dipalmitoylphosphatidylcholin

180 Absence of SP-C caused a severe progressive pulmonary disorder with

181 on of the monolayer and the concentration of SP-C or SP-C peptide.

182 s designed to identify the peptide domain of SP-C required for sorting and secretion of this integral

183 s been analyzed under the combined effect of SP-C and cholesterol by deuterium NMR and phosphorus NMR

184 g of bilayers, indicating that the effect of SP-C to mobilize cholesterol could be indirectly associa

185 ell phenotype, as evidenced by expression of SP-C and osmiophilic lamellar bodies.

186 Transient expression of SP-C(Deltaexon4) in isolated type II epithelial cells or

187 ss imposed by the constitutive expression of SP-C(Deltaexon4) via an NF-kappaB-dependent pathway.

188 e no single deletion abrogated expression of SP-C, deleting both FGF7 and FGF1 inhibited growth and p

189 ion of exon4, generating a truncated form of SP-C (SP-C(Deltaexon4)).

190 has been found that the alpha-helix form of SP-C is metastable, and under certain circumstances may

191 C-terminus of the hydrophobic alpha-helix of SP-C, where a strictly conserved heptapeptide sequence i

192 patterning, helicity, and hydrophobicity of SP-C, and to include no, one, or two vicinal amide-linke

193 oSP-C) is required for membrane insertion of SP-C and has anti-amyloid activity in vitro.

194 e to Sftpc+/+ mice indicate that the lack of SP-C promotes proinflammatory responses in the lung.

195 n-treated mice with I3C reduced the level of SP-C, L-plastin, annexin A1, and haptoglobin to that of

196 lar bodies, and greatly diminished levels of SP-C mature peptide; in contrast, lung structure and fun

197 founder mice were identified, and a line of SP-C/p53-273H transgenic mice was established from one o

198 SP-C mRNA was not detected in the lungs of SP-C (-/-) mice, nor was mature SP-C protein detected by

199 Many of SP-C's key biophysical properties derive from its highly

200 To develop a non-natural, bioactive mimic of SP-C and to investigate the effects of side chain chemis

201 However, creating an accurate mimic of SP-C that retains its biophysical surface activity is ex

202 Palmitoylation of SP-C had an indirect effect on the extent of protein-lip

203 r columnar cells lined the lung periphery of SP-C/HFH-4 transgenic mice.

204 leucine residue and may hinder processing of SP-C precursor protein.

205 ormal lung structure, complete processing of SP-C proprotein, well formed lamellar bodies, and normal

206 is of proSP-C21, synthesis and processing of SP-C was evaluated using a lung epithelial cell line (A5

207 s indicate that the N-terminal propeptide of SP-C is required for intracellular sorting and secretion

208 and conformation in the N-terminal region of SP-C and may thus permit the peptide to remain in the fi

209 To discern the role of SP-C in lung function, SP-C-deficient (-/-) mice were pr

210 echanisms implicated in the emerging role of SP-C mutations in the pathophysiology of diffuse parench

211 d for intracellular sorting and secretion of SP-C.

212 olar epithelial cells, the cellular sites of SP-C-hCFTR+/+ transgene expression.

213 This review examines the current state of SP-C biosynthesis with a focus on recent developments re

214 to have a role in sorting and trafficking of SP-C and may also be important to the surfactant functio

215 ld type proSP-C to facilitate trafficking of SP-C mutants with intact transmembrane domains but lacki

216 characterize the pulmonary transcriptome of SP-C/TNF-alpha mice and wild-type littermates.

217 The current understanding of SP-C biosynthesis considers the SP-C proprotein (proSP-C

218 he importance of a detailed understanding of SP-C biosynthesis has been the recent association of mut

219 Four-month-old SP-C/TNF-alpha mice displayed pronounced pulmonary infla

220 -specific protein SP-C and peptides based on SP-C eliminate the loss to the subphase of unsaturated l

221 owed that Erm by itself had little effect on SP-C promoter activity but that Erm significantly enhanc

222 In the absence of SP-B or SP-C, the unsaturated lipids are irreversibly lost at hi

223 e monolayer and the concentration of SP-C or SP-C peptide.

224 is markedly deficient in SPs, in particular SP-C.

225 ncoding for either wild type proSP-C (pcDNA3/SP-C-(1-194)) or heterologous fusion proteins containing

226 fluorescence microscopy revealed that pcDNA3/SP-C-(1-194) and EGFP/SP-C-(1-194) were each expressed i

227 When co-transfected with pcDNA3/SP-C-(1-194), both EGFP/SP-C-(24-194) and EGFP/SP-C-(24-

228 not corrected by cotransfection with pcDNA3/SP-C-(1-194).

229 d in vitro testing of two classes of peptoid SP-C mimics: those having a rigid alpha-chiral aromatic

230 FGF7 and FGF1 inhibited growth and prevented SP-C expression.

231 ecombinant BRICHOS domains from Bri2 and pro-SP-C prevent fibril formation of amyloid beta-peptides (

232 ipheral lung tubules, including SP-B and pro-SP-C, was inhibited.

233 together with the linker region, during pro-SP-C biosynthesis in the ER.

234 roblem is to include a BRICHOS domain in pro-SP-C, which functions as a chaperone for SP-C during bio

235 reversal of transmembrane orientation of pro-SP-C and total abrogation of post-translational processi

236 these residues influence modification of pro-SP-C by directing transmembrane orientation.

237 tudies have shown that palmitoylation of pro-SP-C is dependent on two N-terminal juxtamembrane positi

238 umen can interact with the TM segment of pro-SP-C, we studied the membrane insertion properties of th

239 nce on the trafficking and processing of pro-SP-C.

240 ibrosis (surfactant protein C precursor; pro-SP-C) and familial dementia (Bri2).

241 s a 21-kDa integral membrane propeptide (pro-SP-C) and proteolytically processed to a 3.7-kDa secreto

242 ons in the gene of the precursor protein pro-SP-C.

243 roperties of the recombinant form of the pro-SP-C BRICHOS domain and two ILD-associated mutants.

244 Mutations in the pro-SP-C BRICHOS domain or linker region lead to amyloid for

245 Furthermore, to understand how the pro-SP-C BRICHOS domain present in the ER lumen can interact

246 dy translocon-mediated folding of the WT pro-SP-C poly-Val and a designed poly-Leu transmembrane (TM)

247 t the co-translational folding of the WT pro-SP-C TM segment is inefficient, that the BRICHOS domain

248 ning for proSP-C and/or aberrantly processed SP-C was observed in lungs of all infants with SP-B gene

249 the lung under control of the SP-C promoter (SP-C-GM mice) were placed in >95% oxygen.

250 lung-specific surfactant protein C promoter (SP-C-rtTA), to inhibit FGF activity at various times in

251 l of the surfactant protein (SP)-C promoter (SP-C/TNF-alpha-transgenic mice) were sensitized to ovalb

252 om the surfactant protein C (SP-C) promoter (SP-C-hCFTR+/-).

253 synthesized as a 194-amino acid propeptide (SP-C-(1-194)) that is directed to the distal secretory p

254 Lung surfactant-specific protein SP-C and peptides based on SP-C eliminate the loss to th

255 owever, the lung surfactant specific protein SP-C enhances the resistance to surfactant flow by regul

256 The proximal SP-C promoter region contains critical binding sites for

257 e secretion of TGF-beta1 induced by two rare SP-C mutant proteins (I73T and M71V), is largely unaffec

258 ntaining either the full-length cDNA for rat SP-C (SP-Cwt) or one of six polymerase chain reaction (P

259 resonances of porcine and human recombinant SP-C with Met32 replaced by isoleucine point to a dimeri

260 tion of one of the Ets binding sites reduced SP-C transcription to background levels, whereas mutatio

261 teract with TTF-1 to differentially regulate SP-C transcription, we performed transient transfection

262 transfection of HPD mutants failed to rescue SP-C endoplasmic reticulum-associated protein degradatio

263 l mutant (-/-) mice, using the lung-specific SP-C promoter.

264 f protein-lipid perturbations by stabilizing SP-C structure, and seemed to be important to maximize d

265 ion analyses of these regions indicated that SP-C adopts a variety of secondary structure conformatio

266 was decreased significantly, indicating that SP-C plays a role in the stabilization of surfactant at

267 F0 transgene-positive fetuses revealed that SP-C(Deltaexon4) caused a dose-dependent disruption in b

268 Our results show that SP-C induces phase segregation at 37 degrees C, resultin

269 The SP-C (-/-) mice were viable at birth and grew normally t

270 The SP-C minimal promoter was found to contain two potential

271 The SP-C-Ttf1 transgene did not rescue the severe pulmonary

272 The SP-C/p53-273H transgenic mice described here thus repres

273 rstanding of SP-C biosynthesis considers the SP-C proprotein (proSP-C) as a hybrid molecule that inco

274 of captive bubbles with surfactant from the SP-C (-/-) mice was decreased significantly, indicating

275 okines were not substantially altered in the SP-C (-/-) mice.

276 lveolar epithelial barrier properties in the SP-C-GM mice.

277 The atypical epithelial cells seen in the SP-C/HFH-4 mice expressed thyroid transcription factor-1

278 surface pressures, all conformations of the SP-C molecule reacted identically to increasing surface

279 erexpressed in the lung under control of the SP-C promoter (SP-C-GM mice) were placed in >95% oxygen.

280 TTF-1 induced synergistic activation of the SP-C promoter that was further enhanced by p300.

281 28 G --> A mutation causes misfolding of the SP-C proprotein with subsequent induction of the unfolde

282 nker region lead to amyloid formation of the SP-C protein and ILD.

283 he wild-type mice had died, while 70% of the SP-C-GM mice remained alive after 10 days in hyperoxia.

284 ngth of DOX exposure and the presence of the SP-C-rtTA transgene contributed more than previously app

285 produced by expression of high levels of the SP-C/HFH-4 transgene.

286 the transcriptional activity of TTF-1 on the SP-C promoter.

287 We show that the SP-C-driven small hairpin RNAs specifically depress the

288 Addition of SP-A to the SP-C surfactant did not change lung function.

289 dels identified a set of genes unique to the SP-C/TNF-alpha model and revealed that lack of extracell

290 enotypes reported by investigators using the SP-C-rtTA, (tetO)(7)-Cre.

291 ion of two SP-A1 (R219W and R242*) and three SP-C (I73T, M71V, and L188Q) variant proteins lead to th

292 Thus, SP-C functions to limit lung inflammation, inhibit colla

293 fic interactions that could be attributed to SP-C or to the other hydrophobic surfactant protein SP-B

294 g green fluorescent protein (EGFP) linked to SP-C-(1-194) (EGFP/SP-C-(1-194)), to mutant proSP-C lack

295 of the reverse tetracycline transactivator (SP-C-rtTA) enabled functional analysis of essential gene

296 Computational docking of two SP-C helices, described here, reveals a dimer with a hel

297 to facilitate cholesterol dynamics, whereas SP-C does not seem to establish a direct interaction wit

298 To determine whether SP-C expression is regulated by NFI in vivo, a chimeric

299 lesterol could be indirectly associated with SP-C-mediated membrane remodeling.

300 eterotypic oligomerization of wild type with SP-C folding mutants produces a dominant negative thus c