John Rosendahl Østergaard

Cardiac pathology in neuronal ceroid lipofuscinoses (NCL): More than a mere co-morbidity

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperReviewResearchpeer-review

  • Katja Rietdorf, School of Life, Health and Chemical Sciences, The Open University, Walton Hall, Milton Keynes MK7 6AA, UK. Electronic address: katja.rietdorf@open.ac.uk.
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  • Emily E Coode, School of Life, Health and Chemical Sciences, The Open University, Walton Hall, Milton Keynes MK7 6AA, UK.
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  • Angela Schulz, Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
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  • Eva Wibbeler, Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
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  • Martin D Bootman, School of Life, Health and Chemical Sciences, The Open University, Walton Hall, Milton Keynes MK7 6AA, UK.
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  • John R Ostergaard

The neuronal ceroid lipofuscinoses (NCLs) are mostly seen as diseases affecting the central nervous system, but there is accumulating evidence that they have co-morbidities outside the brain. One of these co-morbidities is a decline in cardiac function. This is becoming increasingly recognised in teenagers and adolescents with juvenile CLN3, but it may also occur in individuals with other NCLs. The purpose of this review is to summarise the current knowledge of the structural and functional changes found in the hearts of animal models and people diagnosed with NCL. In addition, we present evidence of structural changes that were observed in a systematic comparison of the cardiomyocytes from CLN3Δex7/8 mice.

Original languageEnglish
Article number165643
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1866
Issue9
ISSN0925-4439
DOIs
Publication statusPublished - 2020

    Research areas

  • Batten disease, Cardiac pathology, Cardiomyocyte structure, ECG, Echocardiogram, Juvenile CLN3

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