CFTR-deficient pigs display peripheral nervous system defects at birth (Q30457535)

15 August 2017

CFTR-deficient pigs display peripheral nervous system defects at birth (English)

1 reference

15 August 2017

Michael S. Hildebrand

8

1 reference

15 August 2017

1

Leah R Reznikov

1 reference

15 August 2017

Qian Dong

2

1 reference

15 August 2017

Jeng-Haur Chen

3

1 reference

15 August 2017

Thomas O Moninger

4

1 reference

15 August 2017

Jung Min Park

5

1 reference

15 August 2017

Yuzhou Zhang

6

1 reference

15 August 2017

Jianyang Du

7

1 reference

15 August 2017

Richard J H Smith

9

1 reference

15 August 2017

Christoph O Randak

10

1 reference

15 August 2017

David A Stoltz

11

1 reference

15 August 2017

Michael J Welsh

12

1 reference

15 August 2017

language of work or name

English

0 references

publication date

4 February 2013

1 reference

Proceedings of the National Academy of Sciences of the United States of America

15 August 2017

1 reference

15 August 2017

110

1 reference

15 August 2017

8

1 reference

15 August 2017

3083-3088

1 reference

Charcot-Marie-Tooth disease and related genetic neuropathies.

15 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Intermediate Charcot-Marie-Tooth disease due to a novel Trp101Stop myelin protein zero mutation associated with debilitating neuropathic pain.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Rare association between cystic fibrosis, Chiari I malformation, and hydrocephalus in a baby: a case report and review of the literature

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

P0S63del impedes the arrival of wild-type P0 glycoprotein to myelin in CMT1B mice

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Calnexin deficiency leads to dysmyelination

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Modular double sucrose gap apparatus for improved recording of compound action potentials from rat and mouse spinal cord white matter preparations

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Development of the enteric nervous system and its role in intestinal motility during fetal and early postnatal stages.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

P2X7-mediated increased intracellular calcium causes functional derangement in Schwann cells from rats with CMT1A neuropathy

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Axonal and periaxonal swelling precede peripheral neurodegeneration in KCC3 knockout mice

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Inflammation and anti-inflammatory therapies for cystic fibrosis

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Impaired gastric myolectrical activity in patients with cystic fibrosis.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Submucosal glands and airway defense

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Discovery of glycine hydrazide pore-occluding CFTR inhibitors: mechanism, structure-activity analysis, and in vivo efficacy

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

New insights into human enteric neuropathies

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Demyelinating and axonal features of Charcot-Marie-Tooth disease with mutations of myelin-related proteins (PMP22, MPZ and Cx32): a clinicopathological study of 205 Japanese patients

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

An axonal form of Charcot-Marie-Tooth disease showing distinctive features in association with mutations in the peripheral myelin protein zero gene (Thr124Met or Asp75Val).

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Vagus nerve stimulation attenuates the systemic inflammatory response to endotoxin

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Novel mutation in the myelin protein zero gene in a family with intermediate hereditary motor and sensory neuropathy

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Axonal phenotype of Charcot-Marie-Tooth disease associated with a mutation in the myelin protein zero gene

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Genetic heterogeneity in autosomal recessive hereditary motor and sensory neuropathy with focally folded myelin sheaths (CMT4B).

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Alterations of annexin expression in pathological neuronal and glial reactions. Immunohistochemical localization of annexins I, II (p36 and p11 subunits), IV, and VI in the human hippocampus

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Cystic fibrosis transmembrane conductance regulator protein expression in brain

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Deficient vasoactive intestinal peptide innervation in the sweat glands of cystic fibrosis patients

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Distal intestinal obstruction syndrome in cystic fibrosis treated by oral intestinal lavage, and a case of recurrent obstruction despite normal pancreatic function

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Autonomic nervous system abnormalities in cystic fibrosis.

20 June 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Cystic fibrosis gene and protein expression during fetal lung development.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Cystic fibrosis transmembrane conductance regulator expression in human spinal and sympathetic ganglia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Autosomal recessive hereditary motor and sensory neuropathy with focally folded myelin sheaths: clinical, electrophysiologic, and genetic aspects of a large family.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Pathology of a mouse mutation in peripheral myelin protein P0 is characteristic of a severe and early onset form of human Charcot-Marie-Tooth type 1B disorder

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

P(0) glycoprotein overexpression causes congenital hypomyelination of peripheral nerves

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Charcot-Marie-Tooth caused by a copy number variation in myelin protein zero.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

The use of pigs in neuroscience: modeling brain disorders.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Effects of clonidine and tricyclic antidepressants on gastric smooth muscle contractility

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Schwann cells and astrocytes induce synapse formation by spinal motor neurons in culture

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.1222729110

Physiological basis of cystic fibrosis: a historical perspective

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3581923

Uncoupling of myelin assembly and schwann cell differentiation by transgenic overexpression of peripheral myelin protein 22

26 November 2018

1 reference

12 December 2020

Abnormal Adrenergic and Cholinergic Sensitivity in Cystic Fibrosis

1 reference

12 December 2020

Neural drive and ventilatory strategy of breathing in normal children, and in patients with cystic fibrosis and asthma

1 reference

12 December 2020

Phenotypic differences between peripheral myelin protein-22 (PMP22) and myelin protein zero (P0) mutations associated with Charcot-Marie-Tooth-related diseases

1 reference

12 December 2020

Increased gene dosage of myelin protein zero causes Charcot-Marie-Tooth disease

1 reference

12 December 2020

Slowly progressive axonal degeneration in a rat model of chronic, nonimmune-mediated demyelination

1 reference

12 December 2020

Effect of vitamin E deficiency on neurologic function in patients with cystic fibrosis

1 reference

12 December 2020

Peripheral nerve dysfunction in patients with cystic fibrosis

1 reference

12 December 2020

Mitogenic factors regulate ion channels in Schwann cells cultured from newborn rat sciatic nerve

1 reference

12 December 2020

Purine release from spinal cord microglia after elevation of calcium by glutamate

1 reference

12 December 2020

Aquagenic wrinkling of the palms in cystic fibrosis and the cystic fibrosis carrier state: a case–control study

1 reference

12 December 2020

Autonomic and airway reactivity in obligate heterozygotes for cystic fibrosis

1 reference

12 December 2020

Peripheral nerve dysfunction in adult patients with cystic fibrosis

1 reference

12 December 2020

Focally folded myelin in Charcot-Marie-Tooth neuropathy type 1B with Ser49Leu in the myelin protein zero.

1 reference

12 December 2020

Somatosensory dysfunction in cystic fibrosis

1 reference

12 December 2020

Impaired brainstem auditory evoked potentials in patients with cystic fibrosis

1 reference

12 December 2020

Mtmr13/Sbf2-deficient mice: an animal model for CMT4B2

1 reference

12 December 2020

Identifiers

DOI

10.1073/PNAS.1222729110

1 reference

15 August 2017

0 references

1 reference

15 August 2017

1 reference