دورية أكاديمية
أعرض في EDS

Kidney organoids generated from erythroid progenitors cells of patients with autosomal dominant polycystic kidney disease.

التفاصيل البيبلوغرافية
العنوان: Kidney organoids generated from erythroid progenitors cells of patients with autosomal dominant polycystic kidney disease.
المؤلفون: Facioli R; Nephrology Division-Federal University of São Paulo, São Paulo, Brazil., Lojudice FH; Cell and Molecular Therapy Center (NUCEL), School of Medicine, University of São Paulo, São Paulo, SP, Brazil., Anauate AC; Nephrology Division-Federal University of São Paulo, São Paulo, Brazil., Maquigussa E; Nephrology Division-Federal University of São Paulo, São Paulo, Brazil., Nishiura JL; Nephrology Division-Federal University of São Paulo, São Paulo, Brazil., Heilberg IP; Nephrology Division-Federal University of São Paulo, São Paulo, Brazil., Sogayar MC; Cell and Molecular Therapy Center (NUCEL), School of Medicine, University of São Paulo, São Paulo, SP, Brazil.; Biochemistry Department, Chemistry Institute, University of São Paulo, São Paulo, SP, Brazil., Boim MA; Nephrology Division-Federal University of São Paulo, São Paulo, Brazil.
المصدر: PloS one [PLoS One] 2021 Aug 02; Vol. 16 (8), pp. e0252156. Date of Electronic Publication: 2021 Aug 02 (Print Publication: 2021).
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Public Library of Science Country of Publication: United States NLM ID: 101285081 Publication Model: eCollection Cited Medium: Internet ISSN: 1932-6203 (Electronic) Linking ISSN: 19326203 NLM ISO Abbreviation: PLoS One Subsets: MEDLINE
أسماء مطبوعة: Original Publication: San Francisco, CA : Public Library of Science
مواضيع طبية MeSH: Erythroid Precursor Cells*/metabolism , Erythroid Precursor Cells*/pathology , Induced Pluripotent Stem Cells*/metabolism , Induced Pluripotent Stem Cells*/pathology , Kidney*/metabolism , Kidney*/pathology , Organoids*/metabolism , Organoids*/pathology , Polycystic Kidney, Autosomal Dominant*/metabolism , Polycystic Kidney, Autosomal Dominant*/pathology, Female ; Humans
مستخلص: Background: Kidney organoids have been broadly obtained from commercially available induced pluripotent stem cells (iPSCs); however, it has been a great challenge to efficiently produce renal organoid models from patients with autosomal dominant polycystic kidney disease (ADPKD) that recapitulate both embryogenesis and the mechanisms of cystogenesis.
Methods: Blood erythroid progenitors (EPs) from two ADPKD patients and one healthy donor (HC) was used as a comparative control to normalize the many technical steps for reprogramming EPs and for the organoids generation. EPs were reprogrammed by an episomal vector into iPSCs, which were differentiated into renal tubular organoids and then stimulated by forskolin to induce cysts formation.
Results: iPSCs derived from EPs exhibited all characteristics of pluripotency and were able to differentiate into all three germ layers. 3D tubular organoids were generated from single cells after 28 days in Matrigel. HC and ADPKD organoids did not spontaneously form cysts, but upon forskolin stimulation, cysts-like structures were observed in the ADPKD organoids but not in the HC-derived organoids.
Conclusion: The findings of this study showed that kidney organoids were successfully generated from the blood EP cells of ADPKD patients and a healthy control donor. This approach should contribute as a powerful tool for embryonic kidney development model, which is able to recapitulate the very early pathophysiological mechanisms involved in cytogenesis.
Competing Interests: The authors have declared that no competing interests exist.
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تواريخ الأحداث: Date Created: 20210802 Date Completed: 20211124 Latest Revision: 20211124
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC8328284
DOI: 10.1371/journal.pone.0252156
PMID: 34339420
قاعدة البيانات: MEDLINE
الوصف
تدمد:1932-6203
DOI:10.1371/journal.pone.0252156