Evidence map›Paper›PMID 27252987›Full record

ReviewDrugs of today (Barcelona, Spain : 1998)2016

Lumacaftor/ivacaftor combination for cystic fibrosis patients homozygous for Phe508del-CFTR.

W Zhang, X Zhang, Y H Zhang, D C Strokes, A P Naren

Abstract readReview
PubMed Full text at PMC Publisher
In one paragraph

Review in Drugs of today (Barcelona, Spain : 1998), 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

13 citing papers in PubMed.

  1. Article
  2. Review
  3. Article
  4. Review
  5. Review
  6. Review
  7. Review
  8. A SYK/SHC1 pathway regulates the amount of CFTR in the plasma membrane.Cellular and molecular life sciences : CMLS · 2020
    Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. CFTR-NHERF2-LPA₂ Complex in the Airway and Gut Epithelia.International journal of molecular sciences · 2017
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

5 authors.

W ZhangDepartment of Pediatrics, Physiology, University of Tennessee Health Science Center, and University of Tennessee Cystic Fibrosis Care and Research Center at Le Bonheur Children's Hospital-Methodist University Hospital, Memphis, Tennessee, USA.
X ZhangDepartment of Pediatrics, University of Tennessee Health Science Center, and University of Tennessee Cystic Fibrosis Care and Research Center at Le Bonheur Children's Hospital-Methodist University Hospital, Memphis, Tennessee, USA.
Y H ZhangDepartment of Bioscience Research, University of Tennessee Health Science Center, Memphis, Tennessee, USA.
D C StrokesDepartment of Pediatrics, University of Tennessee Health Science Center, and University of Tennessee Cystic Fibrosis Care and Research Center at Le Bonheur Children's Hospital-Methodist University Hospital, Memphis, Tennessee, USA.
A P NarenDepartment of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. anaren@cchmc.org.

Funding

Inhibition of an apical cAMP transporter (MRP4) in the gut induces diarrheaR01DK080834 · NIDDK · UNIVERSITY OF TENNESSEE HEALTH SCI CTR · PI Anjaparavanda P Naren · 2009 to 2026
$6.8M
LPA2 receptor-containing complexes in regulating secretory diarrheaR01DK093045 · NIDDK · UNIVERSITY OF TENNESSEE HEALTH SCI CTR · PI NAREN, ANJAPARAVANDA P · 2011 to 2019
$3.0M
Characterization of an inhibitory protein complex for cystic fibrosis therapyR01HL123535 · NHLBI · UNIVERSITY OF TENNESSEE HEALTH SCI CTR · PI ZHANG, WEIQIANG · 2014 to 2018
$1.9M
Identifying molecules that augment mutant CFTR activityR01DK058545 · NIDDK · UNIVERSITY OF TENNESSEE HEALTH SCI CTR · PI NAREN, ANJAPARAVANDA P · 2002 to 2006
$1.1M
NHLBI NIH HHS HL123535NHLBI NIH HHS R01 HL123535NIDDK NIH HHS DK080834NIDDK NIH HHS DK093045NIDDK NIH HHS R01 DK058545NIDDK NIH HHS R01 DK080834NIDDK NIH HHS R01 DK093045
6 · The paper itself

Abstract

Cystic fibrosis (CF) is a life-shortening inherited disease caused by the loss or dysfunction of the CF transmembrane conductance regulator (CFTR) channel activity resulting from mutations in the CFTR gene. Phe508del is the most prevalent mutation, with approximately 90% of all CF patients carrying it on at least one allele. Over the past two or three decades, significant progress has been made in understanding the pathogenesis of CF, and in the development of effective CF therapies. The approval of Orkambi® (lumacaftor/ivacaftor) marks another milestone in CF therapeutics development, which, with the advent of personalized medicine, could potentially revolutionize CF care and management. This article reviews the rationale, progress and future direction in the development of lumacaftor/ivacaftor combination to treat CF patients homozygous for the Phe508del-CFTR mutation.

Indexed as

MutationAminophenolsAminopyridinesBenzodioxolesClinical Trials as TopicCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorDrug Therapy, CombinationHumansPrecision MedicineQuinolonesAminophenolsAminopyridinesBenzodioxolesCystic Fibrosis Transmembrane Conductance RegulatorivacaftorlumacaftorQuinolonesCFTRCystic fibrosisLumacaftor/ivacaftorOrkambi®Phe508del

Identifiers

PMID27252987
PMCPMC5495103

What OpenQuestion holds

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Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.