Rietdorf, Katja; Coode, Emily E.; Schulz, Angela; Wibbeler, Eva; Bootman, Martin D. and Ostergaard, John R.
(2019).
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(Accepted Manuscript)
Due to publisher licensing restrictions, this file is not available for public download until 18 December 2020 Click here to request a copy from the OU Author. |
| DOI (Digital Object Identifier) Link: | https://doi.org/10.1016/j.bbadis.2019.165643 |
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| Google Scholar: | Look up in Google Scholar |
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 |
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| 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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