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Generation of four myotonic dystrophy type 1 patient iPSC lines (CBRCULi002-A, CBRCULi003-A, CBRCULi004-A, CBRCULi005-A) and a control (CBRCULi001-A) derived from lymphoblastoids cell lines. | LitMetric

Generation of four myotonic dystrophy type 1 patient iPSC lines (CBRCULi002-A, CBRCULi003-A, CBRCULi004-A, CBRCULi005-A) and a control (CBRCULi001-A) derived from lymphoblastoids cell lines.

Stem Cell Res

CERVO Research Centre, Institut Universitaire en Santé Mentale de Québec, Quebec City, QC G1J 2G3, Canada; Department of Medicine, Faculty of Medicine, Université Laval, Quebec City, QC G1V 0A6, Canada. Electronic address:

Published: March 2023

AI Article Synopsis

  • * Researchers created and studied four adult stem cell lines (iPSCs) from DM1 patients, which have a CTG repeat expansion in the DM1 gene ranging from 1300 to 1600 repeats, along with a control line from a healthy individual.
  • * The stem cell lines exhibit essential features like pluripotency, the ability to differentiate into various cell types, lack of viral vector contamination, normal genetic structure, and appropriate colony shapes.

Article Abstract

Myotonic dystrophy Type 1 (DM1) is a severe inherited neuromuscular disease and is the most prevalent form of muscular dystrophy in adults. DM1 involves not only the striated muscles including skeletal, and cardiac but also other organs such as the eye, brain and gonads. We have generated and characterized 4 adult heterozygous DM1 iPSC lines carrying between 1300 and 1600 CTG repeat expansion in the DM1 protein kinase gene, and a control from an apparently healthy individual. They all show strong pluripotency markers, differentiation capacity, the absence of residual viral vectors as well as normal karyotypes and colony morphologies.

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Source
http://dx.doi.org/10.1016/j.scr.2023.103037DOI Listing

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