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Cells 2018, 7(11), 188; https://doi.org/10.3390/cells7110188

Neuronal Ceroid Lipofuscinoses: Connecting Calcium Signalling through Calmodulin

1
Department of Pathology, Dalhousie University, Halifax, NS B3H 4R2, Canada
2
Department of Biology, University of Toronto Mississauga, Mississauga, ON L5L 1C6, Canada
3
Department of Cell and Systems Biology, University of Toronto, Toronto, ON M5S 3G5, Canada
4
Department of Biology, Trent University, Peterborough, ON K9L 0G2, Canada
Current address: Department of Biology, Trent University, 1600 West Bank Drive, Peterborough, ON K9L 0G2, Canada.
*
Author to whom correspondence should be addressed.
Received: 11 October 2018 / Revised: 24 October 2018 / Accepted: 27 October 2018 / Published: 29 October 2018
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Abstract

Despite the increased focus on the role of calcium in the neuronal ceroid lipofuscinoses (NCLs, also known as Batten disease), links between calcium signalling and the proteins associated with the disease remain to be identified. A central protein in calcium signalling is calmodulin (CaM), which regulates many of the same cellular processes affected in the NCLs. In this study, we show that 11 of the 13 NCL proteins contain putative CaM-binding domains (CaMBDs). Many of the missense mutations documented from NCL patients overlap with the predicted CaMBDs and are often key residues of those domains. The two NCL proteins lacking such domains, CLN7 and CLN11, share a commonality in undergoing proteolytic processing by cathepsin L, which contains a putative CaMBD. Since CaM appears to have both direct and indirect roles in the NCLs, targeting it may be a valid therapeutic approach for treating the disease. View Full-Text
Keywords: batten disease; neuronal ceroid lipofuscinosis; calmodulin; calmodulin-binding proteins; calmodulin-binding domains; calcium batten disease; neuronal ceroid lipofuscinosis; calmodulin; calmodulin-binding proteins; calmodulin-binding domains; calcium
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
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Mathavarajah, S.; O’Day, D.H.; Huber, R.J. Neuronal Ceroid Lipofuscinoses: Connecting Calcium Signalling through Calmodulin. Cells 2018, 7, 188.

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