Article ; Online: Cardiac pathology in neuronal ceroid lipofuscinoses (NCL): More than a mere co-morbidity.
Biochimica et biophysica acta. Molecular basis of disease
2019 Volume 1866, Issue 9, Page(s) 165643
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. ... ...
| 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 CLN3 |
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| MeSH term(s) | Animals ; Disease Models, Animal ; Heart Diseases/diagnosis ; Heart Diseases/metabolism ; Humans ; Membrane Glycoproteins/metabolism ; Molecular Chaperones/metabolism ; Myocytes, Cardiac/metabolism ; Myocytes, Cardiac/pathology ; Neuronal Ceroid-Lipofuscinoses/diagnosis ; Neuronal Ceroid-Lipofuscinoses/metabolism | |||||
| Chemical Substances | CLN3 protein, mouse ; Membrane Glycoproteins ; Molecular Chaperones | |||||
| Language | English | |||||
| Publishing date | 2019-12-19 | |||||
| Publishing country | Netherlands | |||||
| Document type | Journal Article ; Research Support, Non-U.S. Gov't ; Review | |||||
| ZDB-ID | 60-7 | |||||
| ISSN | 1879-260X ; 1879-2596 ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650 ; 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399 | |||||
| ISSN (online) | 1879-260X ; 1879-2596 ; 1872-8006 ; 1879-2642 ; 1879-2618 ; 1879-2650 | |||||
| ISSN | 0006-3002 ; 0005-2728 ; 0005-2736 ; 0304-4165 ; 0167-4838 ; 1388-1981 ; 0167-4889 ; 0167-4781 ; 0304-419X ; 1570-9639 ; 0925-4439 ; 1874-9399 | |||||
| DOI | 10.1016/j.bbadis.2019.165643 | |||||
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| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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