Cardiac pathology in neuronal ceroid lipofuscinoses (NCL): More than a mere co-morbidity.
Batten disease
Cardiac pathology
Cardiomyocyte structure
ECG
Echocardiogram
Juvenile CLN3
Journal
Biochimica et biophysica acta. Molecular basis of disease
ISSN: 1879-260X
Titre abrégé: Biochim Biophys Acta Mol Basis Dis
Pays: Netherlands
ID NLM: 101731730
Informations de publication
Date de publication:
01 09 2020
01 09 2020
Historique:
received:
17
09
2019
revised:
10
12
2019
accepted:
11
12
2019
pubmed:
22
12
2019
medline:
22
12
2020
entrez:
22
12
2019
Statut:
ppublish
Résumé
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 CLN3
Identifiants
pubmed: 31863828
pii: S0925-4439(19)30373-4
doi: 10.1016/j.bbadis.2019.165643
pii:
doi:
Substances chimiques
CLN3 protein, mouse
0
Membrane Glycoproteins
0
Molecular Chaperones
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
165643Informations de copyright
Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of competing interest The authors declare no conflict of interest for this study.