JMUi001-A-1

Registration Summary:

A P E C

DNAJC19tv

The cell line is not submitted yet.

(only basic data is shown)

General

iPSC Line
hPSCreg name	JMUi001-A-1
Cite as:	JMUi001-A-1 (RRID:CVCL_ZL59)
Alternative name(s)	DNAJC19tv
iPSC line type	Human induced pluripotent stem cell (hiPSC)
Similar iPSC lines	No similar lines found.
Last update	3rd April 2020
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Provider
Generator	Comprehensive Heart Failure Center, University Clinic Wuerzburg (UKWCHF)
External iPSC Databases
Cellosaurus	CVCL_ZL59
Wikidata	Q98126798
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.
Subclone of	JMUi001-A

iPSC Derivation

General
The source cell information can be found in the parental cell line JMUi001-A.