(Q36891023)

16 August 2017

Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs (English)

1 reference

intestinal obstruction in the newborn due to guanylate cyclase 2C deficiency

16 August 2017

0 references

1 reference

1 reference

1 reference

Mahmoud H Abou Alaiwa

Mahmoud H Abou Alaiwa

12

0 references

Leah R Reznikov

Leah R Reznikov

8

0 references

Alejandro A Pezzulo

Alejandro A Pezzulo

4

0 references

Lynda S Ostedgaard

Lynda S Ostedgaard

5

0 references

David Stoltz

David A Stoltz

1

0 references

David Meyerholz

David K Meyerholz

29

0 references

Paul B McCray

Paul B McCray

27

0 references

Randall S. Prather

Randall S Prather

28

0 references

Eric A Hoffman

Eric A Hoffman

24

0 references

Joseph Zabner

Joseph Zabner

26

0 references

author name string

Tatiana Rokhlina

2

1 reference

16 August 2017

Sarah E Ernst

3

1 reference

16 August 2017

Philip H Karp

6

1 reference

16 August 2017

Melissa S Samuel

7

1 reference

16 August 2017

Michael V Rector

9

1 reference

16 August 2017

Nicholas D Gansemer

10

1 reference

16 August 2017

Drake C Bouzek

11

1 reference

16 August 2017

Mark J Hoegger

13

1 reference

16 August 2017

Paula S Ludwig

14

1 reference

16 August 2017

Peter J Taft

15

1 reference

16 August 2017

Tanner J Wallen

16

1 reference

16 August 2017

Christine Wohlford-Lenane

17

1 reference

16 August 2017

James D McMenimen

18

1 reference

16 August 2017

Jeng-Haur Chen

19

1 reference

16 August 2017

Katrina L Bogan

20

1 reference

16 August 2017

Ryan J Adam

21

1 reference

16 August 2017

Emma E Hornick

22

1 reference

16 August 2017

George A Nelson

23

1 reference

16 August 2017

Eugene H Chang

25

1 reference

16 August 2017

Michael J Welsh

30

1 reference

16 August 2017

language of work or name

English

0 references

publication date

8 May 2013

1 reference

Journal of Clinical Investigation

16 August 2017

1 reference

16 August 2017

123

1 reference

16 August 2017

6

1 reference

16 August 2017

2685-2693

1 reference

Gastrointestinal complications of cystic fibrosis

16 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Swine models of cystic fibrosis reveal male reproductive tract phenotype at birth

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Issues in the management of simple and complex meconium ileus

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Intestinal obstruction syndromes in cystic fibrosis: meconium ileus, distal intestinal obstruction syndrome, and constipation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Pharmacological correction of a defect in PPAR-gamma signaling ameliorates disease severity in Cftr-deficient mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Pathology of gastrointestinal organs in a porcine model of cystic fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

CFTR delivery to 25% of surface epithelial cells restores normal rates of mucus transport to human cystic fibrosis airway epithelium

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Imaging of the chest in cystic fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Exacerbations in cystic fibrosis. 1: Epidemiology and pathogenesis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Relative contribution of genetic and nongenetic modifiers to intestinal obstruction in cystic fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Generation of cloned transgenic pigs rich in omega-3 fatty acids

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Gene transfer of CFTR to airway epithelia: low levels of expression are sufficient to correct Cl- transport and overexpression can generate basolateral CFTR.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Production of alpha-1,3-galactosyltransferase knockout pigs by nuclear transfer cloning.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Meconium ileus: a ten-year review of thirty-six patients

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Contemporary management of meconium ileus

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Transgenic mice containing intestinal fatty acid-binding protein-human growth hormone fusion genes exhibit correct regional and cell-specific expression of the reporter gene in their small intestine

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Genetic determination of exocrine pancreatic function in cystic fibrosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Intestinal bicarbonate secretion in cystic fibrosis mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Complete activation of porcine oocytes induced by the sulfhydryl reagent, thimerosal.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Tissue specific expression and developmental regulation of two genes coding for rat fatty acid binding proteins.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Localization, function and regulation of the two intestinal fatty acid-binding protein types.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

In vitro development of preimplantation porcine nuclear transfer embryos cultured in different media and gas atmospheres.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Production of cloned pigs by using somatic cells as donors

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Adult cystic fibrosis exacerbations and new strains of Pseudomonas aeruginosa

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Cystic fibrosis transmembrane conductance regulator and Na+ channel subunits mRNA transcripts, and Cl- efflux, show a different distribution in rat duodenum and colon.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

An in vitro model of differentiated human airway epithelia. Methods for establishing primary cultures.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Physiological basis of cystic fibrosis: a historical perspective

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3668832

Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis

4 September 2018

1 reference

12 December 2020

Pulmonary exacerbations in cystic fibrosis

1 reference

12 December 2020

In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator

1 reference

12 December 2020

Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR

1 reference

12 December 2020

Dual role of CFTR in cAMP-stimulated HCO3- secretion across murine duodenum

1 reference

12 December 2020

Meconium ileus secondary to cystic fibrosis. The East London experience

1 reference

12 December 2020

Identifiers

DOI

10.1172/JCI68867

1 reference

16 August 2017

1 reference

16 August 2017

1 reference