LIBUCi001-A

Registration Summary:

A P E C

The cell line is not submitted yet.

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General

iPSC Line
hPSCreg name	LIBUCi001-A
Cite as:	LIBUCi001-A (RRID:CVCL_ZL56)
iPSC line type	Human induced pluripotent stem cell (hiPSC)
Similar iPSC lines	No similar lines found.
Last update	24th March 2020
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Provider
Generator	Libin Cardiovascular Institute of Alberta, University of Calgary (LIBUC)
External iPSC Databases
Cellosaurus	CVCL_ZL56
Wikidata	Q98126911
General iPSC Information
Publications	Janz A et al. Generation of two patient-derived iPSC lines from siblings (LIBUCi001-A and LIBUCi002-A) and a genetically modified iPSC line (JMUi001-A-1) to mimic dilated cardiomyopathy with ataxia (DCMA) caused by a homozygous DNAJC19 mutation. Stem cell research. 2020 Jul;46:101856. Wasmus C et al. Metabolic Alterations Caused by Defective Cardiolipin Remodeling in Inherited Cardiomyopathies. Life (Basel, Switzerland). 2020 Nov 11;10(11). McKnight CL et al. Modelling Mitochondrial Disease in Human Pluripotent Stem Cells: What Have We Learned?. International journal of molecular sciences. 2021 Jul 20;22(14). Kumari D et al. Hsp40s play distinct roles during the initial stages of apolipoprotein B biogenesis. Molecular biology of the cell. 2022 Feb 1;33(2):ar15. Popoiu TA et al. Cardiac Involvement in Mitochondrial Disorders. Current heart failure reports. 2023 Feb;20(1):76-87. Janz A et al. Mutations in DNAJC19 cause altered mitochondrial structure and increased mitochondrial respiration in human iPSC-derived cardiomyocytes. Molecular metabolism. 2024 Jan;79:101859. Maack Christoph et al. Mitochondrial cardiomyopathies: pathogenesis, diagnosis, and treatment. European Heart Journal. 2025-10-22.

iPSC Derivation

General