Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Can Be Improved by Loweri...
Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Can Be Improved by Lowering Accumulation of Fatty Acid Oxidation Intermediates
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Author / Creator
Knottnerus, Suzan J G , Mengarelli, Isabella , Wüst, Rob C I , Baartscheer, Antonius , Bleeker, Jeannette C , Coronel, Ruben , Ferdinandusse, Sacha , Guan, Kaomei , IJlst, Lodewijk , Li, Wener , Luo, Xiaojing , Portero, Vincent M , Ulbricht, Ying , Visser, Gepke , Wanders, Ronald J A , Wijburg, Frits A , Verkerk, Arie O , Houtkooper, Riekelt H and Bezzina, Connie R
Publisher
Switzerland: MDPI AG
Journal title
Language
English
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Publication information
Publisher
Switzerland: MDPI AG
Subjects
More information
Scope and Contents
Contents
Patients with very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) can present with life-threatening cardiac arrhythmias. The pathophysiological mechanism is unknown. We reprogrammed fibroblasts from one mildly and one severely affected VLCADD patient, into human induced pluripotent stem cells (hiPSCs) and differentiated these into cardiomyoc...
Alternative Titles
Full title
Electrophysiological Abnormalities in VLCAD Deficient hiPSC-Cardiomyocytes Can Be Improved by Lowering Accumulation of Fatty Acid Oxidation Intermediates
Authors, Artists and Contributors
Author / Creator
Mengarelli, Isabella
Wüst, Rob C I
Baartscheer, Antonius
Bleeker, Jeannette C
Coronel, Ruben
Ferdinandusse, Sacha
Guan, Kaomei
IJlst, Lodewijk
Li, Wener
Luo, Xiaojing
Portero, Vincent M
Ulbricht, Ying
Visser, Gepke
Wanders, Ronald J A
Wijburg, Frits A
Verkerk, Arie O
Houtkooper, Riekelt H
Bezzina, Connie R
Identifiers
Primary Identifiers
Record Identifier
TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_7177397
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_7177397
Other Identifiers
ISSN
1422-0067,1661-6596
E-ISSN
1422-0067
DOI
10.3390/ijms21072589
