Generation and characterization of a delta F508 cystic fibrosis mouse model. (Q52207066)

27 April 2018

title

Generation and characterization of a delta F508 cystic fibrosis mouse model. (English)

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27 April 2018

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2

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author name string

Colledge WH

1

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27 April 2018

Southern KW

3

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27 April 2018

Ratcliff R

4

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27 April 2018

Jiang C

5

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27 April 2018

Cheng SH

6

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27 April 2018

MacVinish LJ

7

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27 April 2018

Anderson JR

8

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Cuthbert AW

9

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Evans MJ

10

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language of work or name

English

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publication date

1 August 1995

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27 April 2018

published in

Nature Genetics

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27 April 2018

volume

10

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27 April 2018

page(s)

445-452

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27 April 2018

issue

4

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https://scigraph.springernature.com/pub.10.1038/ng0895-445

exact match

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cites work

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Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

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Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

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The cystic fibrosis mutation (delta F508) does not influence the chloride channel activity of CFTR.

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Non-invasive liposome-mediated gene delivery can correct the ion transport defect in cystic fibrosis mutant mice

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Differential acidic pH sensitivity of delta F508 CFTR Cl- channel activity in lipid bilayers.

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Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus Oocytes

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Extensive posttranscriptional deletion of the coding sequences for part of nucleotide-binding fold 1 in respiratory epithelial mRNA transcripts of the cystic fibrosis transmembrane conductance regulator gene is not associated with the clinical manif

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Long-term survival of the exon 10 insertional cystic fibrosis mutant mouse is a consequence of low level residual wild-type Cftr gene expression.

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Identifiers

DOI

10.1038/NG0895-445

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27 April 2018

PubMed ID

7545494

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27 April 2018