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

Rietdorf, Katja; Coode, Emily E.; Schulz, Angela; Wibbeler, Eva; Bootman, Martin D. and Ostergaard, John R. (2019). Cardiac pathology in neuronal ceroid lipofuscinoses (NCL): More than a mere co-morbidity. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, article no. 165643.

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DOI (Digital Object Identifier) Link: https://doi.org/10.1016/j.bbadis.2019.165643
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Abstract

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.

Item Type: Journal Item
ISSN: 0925-4439
Academic Unit/School: Faculty of Science, Technology, Engineering and Mathematics (STEM) > Life, Health and Chemical Sciences
Faculty of Science, Technology, Engineering and Mathematics (STEM)
Item ID: 68607
Depositing User: ORO Import
Date Deposited: 20 Dec 2019 13:58
Last Modified: 27 Jun 2020 01:08
URI: http://oro.open.ac.uk/id/eprint/68607
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