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Int. J. Mol. Sci. 2018, 19(6), 1714; https://doi.org/10.3390/ijms19061714

Molecular Genetics of Frontotemporal Dementia Elucidated by Drosophila Models—Defects in Endosomal–Lysosomal Pathway

Department of Biology, Colby College, 5720 Mayflower Hill, Waterville, ME 04901, USA
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Received: 19 May 2018 / Revised: 6 June 2018 / Accepted: 7 June 2018 / Published: 9 June 2018
(This article belongs to the Special Issue Drosophila Model and Human Disease)
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Abstract

Frontotemporal dementia (FTD) is the second most common senile neurodegenerative disease. FTD is a heterogeneous disease that can be classified into several subtypes. A mutation in CHMP2B locus (CHMP2Bintron5), which encodes a component of endosomal sorting complex required for transport-III (ESCRT-III), is associated with a rare hereditary subtype of FTD linked to chromosome 3 (FTD-3). ESCRT is involved in critical cellular processes such as multivesicular body (MVB) formation during endosomal–lysosomal pathway and autophagy. ESCRT mutants causes diverse physiological defects primarily due to accumulation of endosomes and defective MVBs resulting in misregulation of signaling pathways. Charged multivesicular body protein 2B (CHMP2B) is important for neuronal physiology which especially rely on precise regulation of protein homeostasis due to their post-mitotic status. Drosophila has proven to be an excellent model for charaterization of mechanistic underpinning of neurodegenerative disorders including FTD. In this review, current understanding of various FTD-related mutations is discussed with a focus on Drosophila models of CHMP2Bintron5-associated FTD. View Full-Text
Keywords: frontotemporal dementia; endosomal-lysosomal pathway; ESCRT; CHMP2B; CHMP2Bintron5; animal models; Drosophila frontotemporal dementia; endosomal-lysosomal pathway; ESCRT; CHMP2B; CHMP2Bintron5; animal models; Drosophila
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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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Vandal, S.E.; Zheng, X.; Ahmad, S.T. Molecular Genetics of Frontotemporal Dementia Elucidated by Drosophila Models—Defects in Endosomal–Lysosomal Pathway. Int. J. Mol. Sci. 2018, 19, 1714.

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