Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation

Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation

By using a nonintegrating plasmid delivery system, we generated induced pluripotent stem cells (iPSCs) from the urine cells of a male patient from the family carrying the TMC1 p.M418K mutation. This mutation is homologous to that in Beethoven mice, which were treated by gene editing successfully. Th...

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Main Authors: Hongyang Wang, Kaiwen Wu, Jing Guan, Qiuju Wang
Format: Article
Language:English
Published: Elsevier 2020-12-01
Series:Stem Cell Research
Online Access:http://www.sciencedirect.com/science/article/pii/S187350612030283X
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spelling doaj-6a271c7bd3b94f9a9078bb565d7e97422020-12-19T05:04:44ZengElsevierStem Cell Research1873-50612020-12-0149101982Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutationHongyang Wang0Kaiwen Wu1Jing Guan2Qiuju Wang3College of Otolaryngology, Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, 100853 Beijing, China; National Clinical Research Center for Otolaryngologic Diseases, China; Key Lab of Hearing Impairment Science of Ministry of Education, China; Key Lab of Hearing Impairment Prevention and Treatment of Beijing, ChinaCollege of Otolaryngology, Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, 100853 Beijing, China; National Clinical Research Center for Otolaryngologic Diseases, China; Key Lab of Hearing Impairment Science of Ministry of Education, China; Key Lab of Hearing Impairment Prevention and Treatment of Beijing, ChinaCollege of Otolaryngology, Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, 100853 Beijing, China; National Clinical Research Center for Otolaryngologic Diseases, China; Key Lab of Hearing Impairment Science of Ministry of Education, China; Key Lab of Hearing Impairment Prevention and Treatment of Beijing, ChinaCollege of Otolaryngology, Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, 100853 Beijing, China; National Clinical Research Center for Otolaryngologic Diseases, China; Key Lab of Hearing Impairment Science of Ministry of Education, China; Key Lab of Hearing Impairment Prevention and Treatment of Beijing, China; Corresponding author at: College of Otolaryngology, Head and Neck Surgery, Chinese PLA Institute of Otolaryngology, Chinese PLA General Hospital, 28 Fuxing Road, 100853 Beijing, China.By using a nonintegrating plasmid delivery system, we generated induced pluripotent stem cells (iPSCs) from the urine cells of a male patient from the family carrying the TMC1 p.M418K mutation. This mutation is homologous to that in Beethoven mice, which were treated by gene editing successfully. The resulting iPSCs had a normal karyotype, showed pluripotency by immunofluorescence staining, and differentiated into the three germ layers in vivo. This cellular model will provide a useful platform for investigating the pathogenic mechanisms of TMC1-related deafness, further laying the foundation for clinical transformation applications and providing a reference for the final gene therapy in humans.http://www.sciencedirect.com/science/article/pii/S187350612030283X
collection DOAJ
language English
format Article
sources DOAJ
author Hongyang Wang
Kaiwen Wu
Jing Guan
Qiuju Wang
spellingShingle Hongyang Wang
Kaiwen Wu
Jing Guan
Qiuju Wang
Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation
Stem Cell Research
author_facet Hongyang Wang
Kaiwen Wu
Jing Guan
Qiuju Wang
author_sort Hongyang Wang
title Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation
title_short Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation
title_full Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation
title_fullStr Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation
title_full_unstemmed Generation of a human induced pluripotent stem cell line (CPGHi001-A) from a hearing loss patient with the TMC1 p.M418K mutation
title_sort generation of a human induced pluripotent stem cell line (cpghi001-a) from a hearing loss patient with the tmc1 p.m418k mutation
publisher Elsevier
series Stem Cell Research
issn 1873-5061
publishDate 2020-12-01
description By using a nonintegrating plasmid delivery system, we generated induced pluripotent stem cells (iPSCs) from the urine cells of a male patient from the family carrying the TMC1 p.M418K mutation. This mutation is homologous to that in Beethoven mice, which were treated by gene editing successfully. The resulting iPSCs had a normal karyotype, showed pluripotency by immunofluorescence staining, and differentiated into the three germ layers in vivo. This cellular model will provide a useful platform for investigating the pathogenic mechanisms of TMC1-related deafness, further laying the foundation for clinical transformation applications and providing a reference for the final gene therapy in humans.
url http://www.sciencedirect.com/science/article/pii/S187350612030283X
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