Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length

Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length

Werner syndrome (WS) is a rare human autosomal recessive disorder characterized by early onset of aging-associated diseases, chromosomal instability, and cancer predisposition, without therapeutic treatment solution. Major clinical symptoms of WS include common age-associated diseases, such as insul...

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Main Authors: Vincent Gatinois, Romain Desprat, Fabienne Becker, Lydiane Pichard, Florence Bernex, Carole Corsini, Franck Pellestor, Jean-Marc Lemaitre
Format: Article
Language:English
Published: Elsevier 2019-08-01
Series:Stem Cell Research
Online Access:http://www.sciencedirect.com/science/article/pii/S187350611930145X
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spelling doaj-d587a51bddb24841a2f27f0443d42eff2020-11-24T21:49:53ZengElsevierStem Cell Research1873-50612019-08-0139Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere lengthVincent Gatinois0Romain Desprat1Fabienne Becker2Lydiane Pichard3Florence Bernex4Carole Corsini5Franck Pellestor6Jean-Marc Lemaitre7Laboratory of Genome and Stem Cell Plasticity in Development and Aging, Institute for Regenerative Medicine and Biotherapy, INSERM UMR1183, Univ Montpellier, Montpellier, France; Laboratory of Cytogenetics, ChromoStem Facility, Univ Montpellier, CHU de Montpellier, Montpellier, FranceSAFE-iPSC Facility INGESTEM, Univ Montpellier, CHU de Montpellier, Montpellier, FranceSAFE-iPSC Facility INGESTEM, Univ Montpellier, CHU de Montpellier, Montpellier, FranceLaboratory of Genome and Stem Cell Plasticity in Development and Aging, Institute for Regenerative Medicine and Biotherapy, INSERM UMR1183, Univ Montpellier, Montpellier, France; SAFE-iPSC Facility INGESTEM, Univ Montpellier, CHU de Montpellier, Montpellier, FranceInstitut de Recherche en Cancérologie de Montpellier, Univ Montpellier, INSERM, U1194, Montpellier, France; Network of Experimental Histology, Univ Montpellier, BioCampus, CNRS, UMS3426, Montpellier, FranceMedical Genetics Department, Univ Montpellier, CHU de Montpellier, Montpellier, FranceLaboratory of Genome and Stem Cell Plasticity in Development and Aging, Institute for Regenerative Medicine and Biotherapy, INSERM UMR1183, Univ Montpellier, Montpellier, France; Laboratory of Cytogenetics, ChromoStem Facility, Univ Montpellier, CHU de Montpellier, Montpellier, France; SAFE-iPSC Facility INGESTEM, Univ Montpellier, CHU de Montpellier, Montpellier, France; Corresponding author at: Pr. Franck Pellestor Laboratory of Cytogenetics, ChromoStem Facility, Montpellier University Hospital, Montpellier, France.Laboratory of Genome and Stem Cell Plasticity in Development and Aging, Institute for Regenerative Medicine and Biotherapy, INSERM UMR1183, Univ Montpellier, Montpellier, France; SAFE-iPSC Facility INGESTEM, Univ Montpellier, CHU de Montpellier, Montpellier, France; Correspondence to: Lemaitre Jean-Marc, Laboratory of Genome and Stem Cell Plasticity in Development and Aging, Institute for Regenerative Medicine and Biotherapy, INSERM UMR1183, Univ Montpellier, Montpellier, France.Werner syndrome (WS) is a rare human autosomal recessive disorder characterized by early onset of aging-associated diseases, chromosomal instability, and cancer predisposition, without therapeutic treatment solution. Major clinical symptoms of WS include common age-associated diseases, such as insulin-resistant diabetes mellitus, and atherosclerosis. WRN, the gene responsible for the disease, encodes a RECQL-type DNA helicase with a role in telomere metabolism. We derived a stable iPSC line from 53 years old patient's PBMC, with a normal karyotype, but exhibiting a short telomere length, as a major aspect of the cellular phenotype involved in the pathology.http://www.sciencedirect.com/science/article/pii/S187350611930145X
collection DOAJ
language English
format Article
sources DOAJ
author Vincent Gatinois
Romain Desprat
Fabienne Becker
Lydiane Pichard
Florence Bernex
Carole Corsini
Franck Pellestor
Jean-Marc Lemaitre
spellingShingle Vincent Gatinois
Romain Desprat
Fabienne Becker
Lydiane Pichard
Florence Bernex
Carole Corsini
Franck Pellestor
Jean-Marc Lemaitre
Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length
Stem Cell Research
author_facet Vincent Gatinois
Romain Desprat
Fabienne Becker
Lydiane Pichard
Florence Bernex
Carole Corsini
Franck Pellestor
Jean-Marc Lemaitre
author_sort Vincent Gatinois
title Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length
title_short Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length
title_full Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length
title_fullStr Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length
title_full_unstemmed Reprogramming of Human Peripheral Blood Mononuclear Cell (PBMC) from a patient suffering of a Werner syndrome resulting in iPSC line (REGUi003-A) maintaining a short telomere length
title_sort reprogramming of human peripheral blood mononuclear cell (pbmc) from a patient suffering of a werner syndrome resulting in ipsc line (regui003-a) maintaining a short telomere length
publisher Elsevier
series Stem Cell Research
issn 1873-5061
publishDate 2019-08-01
description Werner syndrome (WS) is a rare human autosomal recessive disorder characterized by early onset of aging-associated diseases, chromosomal instability, and cancer predisposition, without therapeutic treatment solution. Major clinical symptoms of WS include common age-associated diseases, such as insulin-resistant diabetes mellitus, and atherosclerosis. WRN, the gene responsible for the disease, encodes a RECQL-type DNA helicase with a role in telomere metabolism. We derived a stable iPSC line from 53 years old patient's PBMC, with a normal karyotype, but exhibiting a short telomere length, as a major aspect of the cellular phenotype involved in the pathology.
url http://www.sciencedirect.com/science/article/pii/S187350611930145X
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