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Cells 2019, 8(2), 115; https://doi.org/10.3390/cells8020115

Recent Insights into NCL Protein Function Using the Model Organism Dictyostelium discoideum

Department of Biology, Trent University, 1600 West Bank Drive, Peterborough, ON K9L 0G2, Canada
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Received: 23 December 2018 / Revised: 26 January 2019 / Accepted: 30 January 2019 / Published: 2 February 2019
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Abstract

The neuronal ceroid lipofuscinoses (NCLs) are a group of devastating neurological disorders that have a global distribution and affect people of all ages. Commonly known as Batten disease, this form of neurodegeneration is linked to mutations in 13 genetically distinct genes. The precise mechanisms underlying the disease are unknown, in large part due to our poor understanding of the functions of NCL proteins. The social amoeba Dictyostelium discoideum has proven to be an exceptional model organism for studying a wide range of neurological disorders, including the NCLs. The Dictyostelium genome contains homologs of 11 of the 13 NCL genes. Its life cycle, comprised of both single-cell and multicellular phases, provides an excellent system for studying the effects of NCL gene deficiency on conserved cellular and developmental processes. In this review, we highlight recent advances in NCL research using Dictyostelium as a biomedical model. View Full-Text
Keywords: Batten disease; neuronal ceroid lipofuscinosis; Dictyostelium discoideum; TPP1/CLN2; CLN3; CLN5; development Batten disease; neuronal ceroid lipofuscinosis; Dictyostelium discoideum; TPP1/CLN2; CLN3; CLN5; development
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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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McLaren, M.D.; Mathavarajah, S.; Huber, R.J. Recent Insights into NCL Protein Function Using the Model Organism Dictyostelium discoideum. Cells 2019, 8, 115.

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