Generation of MERRF patient-derived induced pluripotent stem cell line iMERRF-C7

Dong Liang; Huanran Hu; Tianhui Xu; Yan Wang; Ping Hu; Zhengfeng Xu

doi:10.1016/j.scr.2016.11.008

Generation of MERRF patient-derived induced pluripotent stem cell line iMERRF-C7

Stem Cell Res. 2016 Nov;17(3):616-618. doi: 10.1016/j.scr.2016.11.008. Epub 2016 Nov 5.

Authors

Dong Liang¹, Huanran Hu¹, Tianhui Xu¹, Yan Wang¹, Ping Hu¹, Zhengfeng Xu²

Affiliations

¹ Department of Prenatal Diagnosis, Obstretrics and Gynecology Hospital Affiliated to Nanjing Medical University, Nanjing, 210004, Jiangsu, China.
² Department of Prenatal Diagnosis, Obstretrics and Gynecology Hospital Affiliated to Nanjing Medical University, Nanjing, 210004, Jiangsu, China. Electronic address: njxzf@126.com.

PMID: 27934592
DOI: 10.1016/j.scr.2016.11.008

Abstract

Human iPSC line iMERRF-C7 was generated from PBMCs of a patient with mitochondrial disorder MERRF. Using Sendai virus, the reprogramming factors Oct3/4, Sox2, Klf4, and cMyc were delivered non-integratively. The resulting iPSCs expressed pluripotency markers, could differentiate into the three germ layers in vivo, had normal genomic structure, and retained the disease-causing m.8344 mutation with similar heteroplasmic level.

MeSH terms

Animals
Base Sequence
Cell Line
Cellular Reprogramming
Female
Genetic Loci
Genetic Vectors / genetics
Genetic Vectors / metabolism
Humans
Induced Pluripotent Stem Cells / cytology*
Induced Pluripotent Stem Cells / metabolism
Induced Pluripotent Stem Cells / transplantation
Karyotype
Kruppel-Like Factor 4
Leukocytes, Mononuclear / cytology
MERRF Syndrome / genetics
MERRF Syndrome / pathology
Mice
Mice, Inbred NOD
Mice, SCID
Microscopy, Fluorescence
Polymorphism, Single Nucleotide
Sendai virus / genetics
Sequence Analysis, DNA
Teratoma / pathology
Transcription Factors / genetics
Transcription Factors / metabolism

Substances

KLF4 protein, human
Klf4 protein, mouse
Kruppel-Like Factor 4
Transcription Factors