Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis

<h3>Introduction</h3><p dir="ltr">Cystic fibrosis (CF) is a hereditary autosomal recessive disorder caused by a range of mutations in the CF Transmembrane Conductance Regulator (CFTR) gene. This gene encodes the CFTR protein, which acts as a chloride channel activated by...

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محفوظ في:
التفاصيل البيبلوغرافية
المؤلف الرئيسي: Toqa Darwish (13222230) (author)
مؤلفون آخرون: Azhar Al-Khulaifi (13222233) (author), Menatalla Ali (13222236) (author), Rana Mowafy (13222239) (author), Abdelilah Arredouani (10914455) (author), Suhail A. Doi (9552474) (author), Mohamed M. Emara (9913215) (author)
منشور في: 2022
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author Toqa Darwish (13222230)
author2 Azhar Al-Khulaifi (13222233)
Menatalla Ali (13222236)
Rana Mowafy (13222239)
Abdelilah Arredouani (10914455)
Suhail A. Doi (9552474)
Mohamed M. Emara (9913215)
author2_role author
author
author
author
author
author
author_facet Toqa Darwish (13222230)
Azhar Al-Khulaifi (13222233)
Menatalla Ali (13222236)
Rana Mowafy (13222239)
Abdelilah Arredouani (10914455)
Suhail A. Doi (9552474)
Mohamed M. Emara (9913215)
author_role author
dc.creator.none.fl_str_mv Toqa Darwish (13222230)
Azhar Al-Khulaifi (13222233)
Menatalla Ali (13222236)
Rana Mowafy (13222239)
Abdelilah Arredouani (10914455)
Suhail A. Doi (9552474)
Mohamed M. Emara (9913215)
dc.date.none.fl_str_mv 2022-08-09T03:00:00Z
dc.identifier.none.fl_str_mv 10.1371/journal.pone.0272091
dc.relation.none.fl_str_mv https://figshare.com/articles/journal_contribution/Assessing_the_consistency_of_iPSC_and_animal_models_in_cystic_fibrosis_modelling_A_meta-analysis/25257238
dc.rights.none.fl_str_mv CC BY 4.0
info:eu-repo/semantics/openAccess
dc.subject.none.fl_str_mv Biological sciences
Genetics
Zoology
Biomedical and clinical sciences
Cardiovascular medicine and haematology
Medical biochemistry and metabolomics
Medical biotechnology
Mathematical sciences
Statistics
Cystic Fibrosis (CF)
Autosomal recessive disorder
CFTR gene
CFTR protein
Respiratory disease
Pathogens
Respiratory failure
Liver, pancreas, small bowel
Molecular mechanisms
Disease modeling
Animal models
iPSC (Induced Pluripotent Stem Cells)
Amyotrophic lateral sclerosis (ALS)
Spinal muscular atrophy (SMA)
Parkinson’s disease (PD)
Alzheimer’s disease (AD)
Drug responses
Chloride channel function
dc.title.none.fl_str_mv Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
dc.type.none.fl_str_mv Text
Journal contribution
info:eu-repo/semantics/publishedVersion
text
contribution to journal
description <h3>Introduction</h3><p dir="ltr">Cystic fibrosis (CF) is a hereditary autosomal recessive disorder caused by a range of mutations in the CF Transmembrane Conductance Regulator (CFTR) gene. This gene encodes the CFTR protein, which acts as a chloride channel activated by cyclic AMP (cAMP). This meta-analysis aimed to compare the responsiveness of induced pluripotent stem cells (iPSCs) to cAMP analogues to that of commonly used animal models.</p><h3>Methods</h3><p dir="ltr">Databases searched included PubMed, Scopus, and Medline from inception to January 2020. A total of 8 and 3 studies, respectively, for animal models and iPSCs, were analyzed. Studies were extracted for investigating cAMP-stimulated anion transport by measuring the short circuit current (Isc) of chloride channels in different animal models and iPSC systems We utilized an inverse variance heterogeneity model for synthesis.</p><h3>Results</h3><p dir="ltr">Our analysis showed considerable heterogeneity in the mean Isc value in both animal models and iPSCs studies (compared to their WT counterparts), and both suffer from variable responsiveness based on the nature of the underlying model. There was no clear advantage of one over the other.</p><h3>Conclusions</h3><p dir="ltr">Studies on both animal and iPSCs models generated considerable heterogeneity. Given the potential of iPSC-derived models to study different diseases, we recommend paying more attention to developing reproducible models of iPSC as it has potential if adequately developed.</p><h2>Other Information</h2><p dir="ltr">Published in: PLOS ONE<br>License: <a href="http://creativecommons.org/licenses/by/4.0/" target="_blank">http://creativecommons.org/licenses/by/4.0/</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1371/journal.pone.0272091" target="_blank">https://dx.doi.org/10.1371/journal.pone.0272091</a></p>
eu_rights_str_mv openAccess
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identifier_str_mv 10.1371/journal.pone.0272091
network_acronym_str Manara2
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oai_identifier_str oai:figshare.com:article/25257238
publishDate 2022
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spelling Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysisToqa Darwish (13222230)Azhar Al-Khulaifi (13222233)Menatalla Ali (13222236)Rana Mowafy (13222239)Abdelilah Arredouani (10914455)Suhail A. Doi (9552474)Mohamed M. Emara (9913215)Biological sciencesGeneticsZoologyBiomedical and clinical sciencesCardiovascular medicine and haematologyMedical biochemistry and metabolomicsMedical biotechnologyMathematical sciencesStatisticsCystic Fibrosis (CF)Autosomal recessive disorderCFTR geneCFTR proteinRespiratory diseasePathogensRespiratory failureLiver, pancreas, small bowelMolecular mechanismsDisease modelingAnimal modelsiPSC (Induced Pluripotent Stem Cells)Amyotrophic lateral sclerosis (ALS)Spinal muscular atrophy (SMA)Parkinson’s disease (PD)Alzheimer’s disease (AD)Drug responsesChloride channel function<h3>Introduction</h3><p dir="ltr">Cystic fibrosis (CF) is a hereditary autosomal recessive disorder caused by a range of mutations in the CF Transmembrane Conductance Regulator (CFTR) gene. This gene encodes the CFTR protein, which acts as a chloride channel activated by cyclic AMP (cAMP). This meta-analysis aimed to compare the responsiveness of induced pluripotent stem cells (iPSCs) to cAMP analogues to that of commonly used animal models.</p><h3>Methods</h3><p dir="ltr">Databases searched included PubMed, Scopus, and Medline from inception to January 2020. A total of 8 and 3 studies, respectively, for animal models and iPSCs, were analyzed. Studies were extracted for investigating cAMP-stimulated anion transport by measuring the short circuit current (Isc) of chloride channels in different animal models and iPSC systems We utilized an inverse variance heterogeneity model for synthesis.</p><h3>Results</h3><p dir="ltr">Our analysis showed considerable heterogeneity in the mean Isc value in both animal models and iPSCs studies (compared to their WT counterparts), and both suffer from variable responsiveness based on the nature of the underlying model. There was no clear advantage of one over the other.</p><h3>Conclusions</h3><p dir="ltr">Studies on both animal and iPSCs models generated considerable heterogeneity. Given the potential of iPSC-derived models to study different diseases, we recommend paying more attention to developing reproducible models of iPSC as it has potential if adequately developed.</p><h2>Other Information</h2><p dir="ltr">Published in: PLOS ONE<br>License: <a href="http://creativecommons.org/licenses/by/4.0/" target="_blank">http://creativecommons.org/licenses/by/4.0/</a><br>See article on publisher's website: <a href="https://dx.doi.org/10.1371/journal.pone.0272091" target="_blank">https://dx.doi.org/10.1371/journal.pone.0272091</a></p>2022-08-09T03:00:00ZTextJournal contributioninfo:eu-repo/semantics/publishedVersiontextcontribution to journal10.1371/journal.pone.0272091https://figshare.com/articles/journal_contribution/Assessing_the_consistency_of_iPSC_and_animal_models_in_cystic_fibrosis_modelling_A_meta-analysis/25257238CC BY 4.0info:eu-repo/semantics/openAccessoai:figshare.com:article/252572382022-08-09T03:00:00Z
spellingShingle Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
Toqa Darwish (13222230)
Biological sciences
Genetics
Zoology
Biomedical and clinical sciences
Cardiovascular medicine and haematology
Medical biochemistry and metabolomics
Medical biotechnology
Mathematical sciences
Statistics
Cystic Fibrosis (CF)
Autosomal recessive disorder
CFTR gene
CFTR protein
Respiratory disease
Pathogens
Respiratory failure
Liver, pancreas, small bowel
Molecular mechanisms
Disease modeling
Animal models
iPSC (Induced Pluripotent Stem Cells)
Amyotrophic lateral sclerosis (ALS)
Spinal muscular atrophy (SMA)
Parkinson’s disease (PD)
Alzheimer’s disease (AD)
Drug responses
Chloride channel function
status_str publishedVersion
title Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
title_full Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
title_fullStr Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
title_full_unstemmed Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
title_short Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
title_sort Assessing the consistency of iPSC and animal models in cystic fibrosis modelling: A meta-analysis
topic Biological sciences
Genetics
Zoology
Biomedical and clinical sciences
Cardiovascular medicine and haematology
Medical biochemistry and metabolomics
Medical biotechnology
Mathematical sciences
Statistics
Cystic Fibrosis (CF)
Autosomal recessive disorder
CFTR gene
CFTR protein
Respiratory disease
Pathogens
Respiratory failure
Liver, pancreas, small bowel
Molecular mechanisms
Disease modeling
Animal models
iPSC (Induced Pluripotent Stem Cells)
Amyotrophic lateral sclerosis (ALS)
Spinal muscular atrophy (SMA)
Parkinson’s disease (PD)
Alzheimer’s disease (AD)
Drug responses
Chloride channel function