ABCMdb

PMID: 20487541

Fang D, West RH, Manson ME, Ruddy J, Jiang D, Previs SF, Sonawane ND, Burgess JD, Kelley TJ
Increased plasma membrane cholesterol in cystic fibrosis cells correlates with CFTR genotype and depends on de novo cholesterol synthesis.
Respir Res. 2010 May 20;11:61., [PubMed]

Sentences

No. Mutations Sentence Comment

8 ABCC7 p.Arg117His Results: Membrane cholesterol measurements are elevated in both R117H and ΔF508 mouse nasal epithelium compared to age-matched sibling wt controls demonstrating a genotype correlation to membrane cholesterol detection. Login to comment 29 ABCC7 p.Arg117His Data demonstrate a clear CFTR genotype correlation with ΔF508 CFTR mice exhibiting higher membrane cholesterol content and increased de novo cholesterol synthesis relative to R117H CFTR mice. Login to comment 35 ABCC7 p.Arg117His These cells were grown at 37°C in 95% O2-5% CO2 on Falcon 10 cm diameter tissue culture dishes in LHC-8 Basal Medium (Biofluids Camarillo, CA) with 5% FBS. Mice Mice homozygous for the ΔF508 CFTR mutation were described previously [12], as were mice carrying the R117H CFTR mutation [13]. Login to comment 91 ABCC7 p.Arg117His In order to determine if membrane cholesterol measurement correlates with Cftr genotype, membrane cholesterol was measured in nasal epithelium isolated from mice homozygous for either the R117H (R/ R) or the ΔF508 (ΔF/ΔF) CFTR mutations. Login to comment 98 ABCC7 p.Arg117His WT mice from the ΔF508 colony and the R117H colony were directly compared and no difference in membrane cholesterol measurement was observed. Login to comment 117 ABCC7 p.Arg117His ABCC7 p.Arg117His A, C) Representative traces of membrane cholesterol determination in excised nasal epithelium from Cftr R117H/R117H (R/R) mice and Cftr ΔF508/ΔF508 (ΔF/ΔF) mice, respectively, compared to sibling Cftr +/+ (wt) mice. B, D) Quantification of responses between CftrR/R and sibling Cftr+/+(wt) nasal tissue and CftrΔF/ΔF nasal tissue compared to Cftr+/+ (wt) siblings. Login to comment 122 ABCC7 p.Arg117His E) Representative traces of wt mice from the ΔF508 (ΔF) and R117H colonies. Login to comment 123 ABCC7 p.Arg117His Mean response for wt mice in the ΔF and R117H colonies are 54.5 +/- 0.5 pC and 55.7 +/- 1.5 pC, respectively. Login to comment