The neuronal ceroid lipofuscinosis protein Cln7 functions in the postsynaptic cell to regulate synapse development

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The neuronal ceroid lipofuscinosis protein Cln7 functions in the postsynaptic cell to regulate synapse development. / Connolly, Kyle; O'Hare, Megan; Mohammed, Alamin; Aitchison, Katelyn; Anthoney, Niki; Taylor, Matthew; Stewart, Bryan; Tuxworth, Richard; Tear, Guy.

In: Scientific Reports, Vol. 9, 15592, 30.10.2019.

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Connolly, Kyle ; O'Hare, Megan ; Mohammed, Alamin ; Aitchison, Katelyn ; Anthoney, Niki ; Taylor, Matthew ; Stewart, Bryan ; Tuxworth, Richard ; Tear, Guy. / The neuronal ceroid lipofuscinosis protein Cln7 functions in the postsynaptic cell to regulate synapse development. In: Scientific Reports. 2019 ; Vol. 9.

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@article{bd06b7cc6f3445d1a40ccfa0f56ea1d8,
title = "The neuronal ceroid lipofuscinosis protein Cln7 functions in the postsynaptic cell to regulate synapse development",
abstract = "The neuronal ceroid lipofuscinoses (NCLs) are a group of fatal, monogenic neurodegenerative disorders with an early onset in infancy or childhood. Despite identification of the genes disrupted in each form of the disease, their normal cellular role and how their deficits lead to disease pathology is not fully understood. Cln7, a major facilitator superfamily domain-containing protein, is affected in a late infantile-onset form of NCL. Cln7 is conserved across species suggesting a common function. Here we demonstrate that Cln7 is required for the normal growth of synapses at the Drosophila larval neuromuscular junction. In a Cln7 mutant, synapses fail to develop fully leading to reduced function and behavioral changes with dysregulation of TOR activity. Cln7 expression is restricted to the post-synaptic cell and the protein localizes to vesicles immediately adjacent to the post-synaptic membrane. Our data suggest an involvement for Cln7 in regulating trans-synaptic communication necessary for normal synapse development.",
author = "Kyle Connolly and Megan O'Hare and Alamin Mohammed and Katelyn Aitchison and Niki Anthoney and Matthew Taylor and Bryan Stewart and Richard Tuxworth and Guy Tear",
year = "2019",
month = oct,
day = "30",
doi = "10.1038/s41598-019-51588-w",
language = "English",
volume = "9",
journal = "Scientific Reports",
issn = "2045-2322",
publisher = "Nature Publishing Group",

}

RIS

TY - JOUR

T1 - The neuronal ceroid lipofuscinosis protein Cln7 functions in the postsynaptic cell to regulate synapse development

AU - Connolly, Kyle

AU - O'Hare, Megan

AU - Mohammed, Alamin

AU - Aitchison, Katelyn

AU - Anthoney, Niki

AU - Taylor, Matthew

AU - Stewart, Bryan

AU - Tuxworth, Richard

AU - Tear, Guy

PY - 2019/10/30

Y1 - 2019/10/30

N2 - The neuronal ceroid lipofuscinoses (NCLs) are a group of fatal, monogenic neurodegenerative disorders with an early onset in infancy or childhood. Despite identification of the genes disrupted in each form of the disease, their normal cellular role and how their deficits lead to disease pathology is not fully understood. Cln7, a major facilitator superfamily domain-containing protein, is affected in a late infantile-onset form of NCL. Cln7 is conserved across species suggesting a common function. Here we demonstrate that Cln7 is required for the normal growth of synapses at the Drosophila larval neuromuscular junction. In a Cln7 mutant, synapses fail to develop fully leading to reduced function and behavioral changes with dysregulation of TOR activity. Cln7 expression is restricted to the post-synaptic cell and the protein localizes to vesicles immediately adjacent to the post-synaptic membrane. Our data suggest an involvement for Cln7 in regulating trans-synaptic communication necessary for normal synapse development.

AB - The neuronal ceroid lipofuscinoses (NCLs) are a group of fatal, monogenic neurodegenerative disorders with an early onset in infancy or childhood. Despite identification of the genes disrupted in each form of the disease, their normal cellular role and how their deficits lead to disease pathology is not fully understood. Cln7, a major facilitator superfamily domain-containing protein, is affected in a late infantile-onset form of NCL. Cln7 is conserved across species suggesting a common function. Here we demonstrate that Cln7 is required for the normal growth of synapses at the Drosophila larval neuromuscular junction. In a Cln7 mutant, synapses fail to develop fully leading to reduced function and behavioral changes with dysregulation of TOR activity. Cln7 expression is restricted to the post-synaptic cell and the protein localizes to vesicles immediately adjacent to the post-synaptic membrane. Our data suggest an involvement for Cln7 in regulating trans-synaptic communication necessary for normal synapse development.

U2 - 10.1038/s41598-019-51588-w

DO - 10.1038/s41598-019-51588-w

M3 - Article

C2 - 31666534

VL - 9

JO - Scientific Reports

JF - Scientific Reports

SN - 2045-2322

M1 - 15592

ER -