Neuronal ceroid lipofuscinosis (Batten disease)
22 total papers: 12 primary research articles, 10 review articles
1 book chapter
Members of the Huber lab are bolded
++ Trent University graduate student
+ Trent University undergraduate student
# Equal contribution
Huber RJ. (2023). Recent insights into the networking of CLN genes and proteins in mammalian cells. Journal of Neurochemistry. In press.
Remtulla AAN++, Huber RJ. (2023). The conserved cellular roles of CLN proteins: Novel insights from Dictyostelium discoideum. European Journal of Cell Biology 102(2), 151305. In Special Issue: Cell Biology's Leading Edge - Part 2. doi: 10.1016/j.ejcb.2023.151305
https://www.sciencedirect.com/science/article/pii/S0171933523000201
Kim WD++, Huber RJ. (2022). An altered transcriptome underlies cln5-deficiency phenotypes in Dictyostelium discoideum. Frontiers in Genetics 13, 1045738 plus supplementary data. In Research Topic: Neuronal Ceroid Lipofuscinosis: Molecular Genetics & Epigenetics. doi: 10.3389/fgene.2022.1045738
https://www.frontiersin.org/articles/10.3389/fgene.2022.1045738/full
Yap SQ++, Kim WD++, Huber RJ. (2022). Mfsd8 modulates growth and the early stages of multicellular development in Dictyostelium discoideum. Frontiers in Cell and Developmental Biology 10, 930235 plus supplementary data. In Research Topic: Ion Transporters and Channels in Cellular Pathophysiology. doi: 10.3389/fcell.2022.930235
https://www.frontiersin.org/articles/10.3389/fcell.2022.930235/full
Kim WD++, Wilson-Smillie MLDM++, Thanabalasingam A++, Lefrancois S, Cotman SL, Huber RJ. (2022). Autophagy in the neuronal ceroid lipofuscinoses (Batten disease). Frontiers in Cell and Developmental Biology 10, 812728. In Research Topic: Defective Macroautophagy in Organelle Turnover: From Basic Mechanisms to Human Disease. doi: 10.3389/fcell.2022.812728. Invited.
Huber RJ. (2021). Altered protein secretion in Batten disease. Disease Models & Mechanisms 14(12), dmm049152 plus supplementary data. In Collection: Model systems in human genetics research, Rare Disease Translational Research Using Model Systems. doi: 10.1242/dmm.049152. Invited.
McLaren MD++#, Mathavarajah S+#, Kim WD++, Yap SQ++, Huber RJ. (2021). Aberrant autophagy impacts growth and multicellular development in a Dictyostelium knockout model of CLN5 disease. Frontiers in Cell and Developmental Biology 9, 657406 plus supplementary data. Research Topic: Dictyostelium: A Tractable Cell and Developmental Model in Biomedical Research. doi: 10.3389/fcell.2021.657406.
Yap SQ++#, Mathavarajah S#, Huber RJ. (2021). The converging roles of Batten disease proteins in neurodegeneration and cancer. iScience 24(4), 102337. doi:10.1016/j.isci.2021.102337
Huber RJ, Hughes SM, Liu W, Morgan A, Tuxworth RI, Russell C. (2020). The contribution of multicellular model organisms to neuronal ceroid lipofuscinosis research. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1866(9), 165614. doi:10.1016/j.bbadis.2019.165614
Huber RJ, Mathavarajah S+, Yap SQ++. (2020). Mfsd8 localizes to endocytic compartments and influences the secretion of Cln5 and cathepsin D in Dictyostelium. Cellular Signalling 70, 109572 plus supplementary data. doi:10.1016/j.cellsig.2020.109572
Huber RJ. (2020). Molecular networking in the neuronal ceroid lipofuscinoses: Insights from mammalian models and the social amoeba Dictyostelium discoideum. Journal of Biomedical Science 27(1), 64. doi:10.1186/s12929-020-00653-y
Huber RJ, Mathavarajah S+. (2019). Comparative transcriptomics reveals mechanisms underlying cln3-deficiency phenotypes in Dictyostelium. Cellular Signalling 58, 79-90 plus supplementary data. doi:10.1016/j.cellsig.2019.02.004
McLaren MD++, Mathavarajah S+, Huber RJ. (2019). Recent insights into NCL protein function using the model organism Dictyostelium discoideum. Cells 8(2), 115. doi:10.3390/cells8020115
Mathavarajah S+, McLaren MD++, Huber RJ. (2018). Cln3 function is linked to osmoregulation in a Dictyostelium model of Batten disease. Biochimica et Biophysica Acta (BBA) – Molecular Basis of Disease 1864, 3559-3573 plus supplementary data. doi:10.1016/j.bbadis.2018.08.013
Mathavarajah S+, O’Day DH, Huber RJ. (2018). Neuronal ceroid lipofuscinoses: Connecting calcium signalling through calmodulin. Cells 7(11), 188. doi:10.3390/cells7110188
Huber RJ, Mathavarajah S+. (2018). Secretion and function of Cln5 during the early stages of Dictyostelium development. Biochimica et Biophysica Acta (BBA) – Molecular Cell Research 1865(10), 1437-1450 plus supplementary data. doi:10.1016/j.bbamcr.2018.07.017
Huber RJ, Mathavarajah S+. (2018). Cln5 is secreted and functions as a glycoside hydrolase in Dictyostelium. Cellular Signalling 42, 236-248 plus supplementary data. doi:10.1016/j.cellsig.2017.11.001
Myre MA#, Huber RJ#, O’Day DH#. (2018). Functional Analysis of Proteins Involved in Neurodegeneration Using the Model Organism Dictyostelium: Alzheimer’s, Huntington’s and Batten Disease. Chapter 21, In: Molecular-Genetic and Statistical Techniques for Behavioral and Neural Research, First Edition, Gerlai RT (Editor), Academic Press: Elsevier, San Diego, CA, pp. 491-518. ISBN: 9780128040782 (Invited).
Huber RJ, Myre MA, Cotman SL. (2017). Aberrant adhesion impacts early development in a Dictyostelium model for juvenile neuronal ceroid lipofuscinosis. Cell Adhesion & Migration 11(4), 399-418. doi:10.1080/19336918.2016.1236179
Huber RJ. (2017). Loss of Cln3 impacts protein secretion in the social amoeba Dictyostelium. Cellular Signalling 35, 61-72 plus supplementary data. doi:10.1016/j.cellsig.2017.03.022
Huber RJ. (2016). Using the social amoeba Dictyostelium to study the functions of proteins linked to neuronal ceroid lipofuscinosis. Journal of Biomedical Science 23(1), 83. doi:10.1186/s12929-016-0301-0
Huber RJ, Myre MA, Cotman SL. (2014). Loss of Cln3 function in the social amoeba Dictyostelium discoideum causes pleiotropic effects that are rescued by human CLN3. PLoS One 9(10), e110544 plus supplementary data. doi:10.1371/journal.pone.0110544
22 total papers: 12 primary research articles, 10 review articles
1 book chapter
Members of the Huber lab are bolded
++ Trent University graduate student
+ Trent University undergraduate student
# Equal contribution
Huber RJ. (2023). Recent insights into the networking of CLN genes and proteins in mammalian cells. Journal of Neurochemistry. In press.
Remtulla AAN++, Huber RJ. (2023). The conserved cellular roles of CLN proteins: Novel insights from Dictyostelium discoideum. European Journal of Cell Biology 102(2), 151305. In Special Issue: Cell Biology's Leading Edge - Part 2. doi: 10.1016/j.ejcb.2023.151305
https://www.sciencedirect.com/science/article/pii/S0171933523000201
Kim WD++, Huber RJ. (2022). An altered transcriptome underlies cln5-deficiency phenotypes in Dictyostelium discoideum. Frontiers in Genetics 13, 1045738 plus supplementary data. In Research Topic: Neuronal Ceroid Lipofuscinosis: Molecular Genetics & Epigenetics. doi: 10.3389/fgene.2022.1045738
https://www.frontiersin.org/articles/10.3389/fgene.2022.1045738/full
Yap SQ++, Kim WD++, Huber RJ. (2022). Mfsd8 modulates growth and the early stages of multicellular development in Dictyostelium discoideum. Frontiers in Cell and Developmental Biology 10, 930235 plus supplementary data. In Research Topic: Ion Transporters and Channels in Cellular Pathophysiology. doi: 10.3389/fcell.2022.930235
https://www.frontiersin.org/articles/10.3389/fcell.2022.930235/full
Kim WD++, Wilson-Smillie MLDM++, Thanabalasingam A++, Lefrancois S, Cotman SL, Huber RJ. (2022). Autophagy in the neuronal ceroid lipofuscinoses (Batten disease). Frontiers in Cell and Developmental Biology 10, 812728. In Research Topic: Defective Macroautophagy in Organelle Turnover: From Basic Mechanisms to Human Disease. doi: 10.3389/fcell.2022.812728. Invited.
Huber RJ. (2021). Altered protein secretion in Batten disease. Disease Models & Mechanisms 14(12), dmm049152 plus supplementary data. In Collection: Model systems in human genetics research, Rare Disease Translational Research Using Model Systems. doi: 10.1242/dmm.049152. Invited.
McLaren MD++#, Mathavarajah S+#, Kim WD++, Yap SQ++, Huber RJ. (2021). Aberrant autophagy impacts growth and multicellular development in a Dictyostelium knockout model of CLN5 disease. Frontiers in Cell and Developmental Biology 9, 657406 plus supplementary data. Research Topic: Dictyostelium: A Tractable Cell and Developmental Model in Biomedical Research. doi: 10.3389/fcell.2021.657406.
Yap SQ++#, Mathavarajah S#, Huber RJ. (2021). The converging roles of Batten disease proteins in neurodegeneration and cancer. iScience 24(4), 102337. doi:10.1016/j.isci.2021.102337
Huber RJ, Hughes SM, Liu W, Morgan A, Tuxworth RI, Russell C. (2020). The contribution of multicellular model organisms to neuronal ceroid lipofuscinosis research. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease 1866(9), 165614. doi:10.1016/j.bbadis.2019.165614
Huber RJ, Mathavarajah S+, Yap SQ++. (2020). Mfsd8 localizes to endocytic compartments and influences the secretion of Cln5 and cathepsin D in Dictyostelium. Cellular Signalling 70, 109572 plus supplementary data. doi:10.1016/j.cellsig.2020.109572
Huber RJ. (2020). Molecular networking in the neuronal ceroid lipofuscinoses: Insights from mammalian models and the social amoeba Dictyostelium discoideum. Journal of Biomedical Science 27(1), 64. doi:10.1186/s12929-020-00653-y
Huber RJ, Mathavarajah S+. (2019). Comparative transcriptomics reveals mechanisms underlying cln3-deficiency phenotypes in Dictyostelium. Cellular Signalling 58, 79-90 plus supplementary data. doi:10.1016/j.cellsig.2019.02.004
McLaren MD++, Mathavarajah S+, Huber RJ. (2019). Recent insights into NCL protein function using the model organism Dictyostelium discoideum. Cells 8(2), 115. doi:10.3390/cells8020115
Mathavarajah S+, McLaren MD++, Huber RJ. (2018). Cln3 function is linked to osmoregulation in a Dictyostelium model of Batten disease. Biochimica et Biophysica Acta (BBA) – Molecular Basis of Disease 1864, 3559-3573 plus supplementary data. doi:10.1016/j.bbadis.2018.08.013
Mathavarajah S+, O’Day DH, Huber RJ. (2018). Neuronal ceroid lipofuscinoses: Connecting calcium signalling through calmodulin. Cells 7(11), 188. doi:10.3390/cells7110188
Huber RJ, Mathavarajah S+. (2018). Secretion and function of Cln5 during the early stages of Dictyostelium development. Biochimica et Biophysica Acta (BBA) – Molecular Cell Research 1865(10), 1437-1450 plus supplementary data. doi:10.1016/j.bbamcr.2018.07.017
Huber RJ, Mathavarajah S+. (2018). Cln5 is secreted and functions as a glycoside hydrolase in Dictyostelium. Cellular Signalling 42, 236-248 plus supplementary data. doi:10.1016/j.cellsig.2017.11.001
Myre MA#, Huber RJ#, O’Day DH#. (2018). Functional Analysis of Proteins Involved in Neurodegeneration Using the Model Organism Dictyostelium: Alzheimer’s, Huntington’s and Batten Disease. Chapter 21, In: Molecular-Genetic and Statistical Techniques for Behavioral and Neural Research, First Edition, Gerlai RT (Editor), Academic Press: Elsevier, San Diego, CA, pp. 491-518. ISBN: 9780128040782 (Invited).
Huber RJ, Myre MA, Cotman SL. (2017). Aberrant adhesion impacts early development in a Dictyostelium model for juvenile neuronal ceroid lipofuscinosis. Cell Adhesion & Migration 11(4), 399-418. doi:10.1080/19336918.2016.1236179
Huber RJ. (2017). Loss of Cln3 impacts protein secretion in the social amoeba Dictyostelium. Cellular Signalling 35, 61-72 plus supplementary data. doi:10.1016/j.cellsig.2017.03.022
Huber RJ. (2016). Using the social amoeba Dictyostelium to study the functions of proteins linked to neuronal ceroid lipofuscinosis. Journal of Biomedical Science 23(1), 83. doi:10.1186/s12929-016-0301-0
Huber RJ, Myre MA, Cotman SL. (2014). Loss of Cln3 function in the social amoeba Dictyostelium discoideum causes pleiotropic effects that are rescued by human CLN3. PLoS One 9(10), e110544 plus supplementary data. doi:10.1371/journal.pone.0110544
