Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment

Batten disease is unique among lysosomal storage disorders for the early and profound manifestation in the central nervous system, but little is known regarding potential neuron-specific roles for the disease-associated proteins. We demonstrate substantial overlap in the protein interactomes of thre...

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Autores principales: Rechtzigel, Mitchell J., Meyerink, Brandon L., Leppert, Hannah, Johnson, Tyler B., Cain, Jacob T., Ferrandino, Gavin, May, Danielle G., Roux, Kyle J., Brudvig, Jon J., Weimer, Jill M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9174988/
https://www.ncbi.nlm.nih.gov/pubmed/35692423
http://dx.doi.org/10.3389/fnins.2022.834780
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author Rechtzigel, Mitchell J.
Meyerink, Brandon L.
Leppert, Hannah
Johnson, Tyler B.
Cain, Jacob T.
Ferrandino, Gavin
May, Danielle G.
Roux, Kyle J.
Brudvig, Jon J.
Weimer, Jill M.
author_facet Rechtzigel, Mitchell J.
Meyerink, Brandon L.
Leppert, Hannah
Johnson, Tyler B.
Cain, Jacob T.
Ferrandino, Gavin
May, Danielle G.
Roux, Kyle J.
Brudvig, Jon J.
Weimer, Jill M.
author_sort Rechtzigel, Mitchell J.
collection PubMed
description Batten disease is unique among lysosomal storage disorders for the early and profound manifestation in the central nervous system, but little is known regarding potential neuron-specific roles for the disease-associated proteins. We demonstrate substantial overlap in the protein interactomes of three transmembrane Batten proteins (CLN3, CLN6, and CLN8), and that their absence leads to synaptic depletion of key partners (i.e., SNAREs and tethers) and altered synaptic SNARE complexing in vivo, demonstrating a novel shared etiology.
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spelling pubmed-91749882022-06-09 Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment Rechtzigel, Mitchell J. Meyerink, Brandon L. Leppert, Hannah Johnson, Tyler B. Cain, Jacob T. Ferrandino, Gavin May, Danielle G. Roux, Kyle J. Brudvig, Jon J. Weimer, Jill M. Front Neurosci Neuroscience Batten disease is unique among lysosomal storage disorders for the early and profound manifestation in the central nervous system, but little is known regarding potential neuron-specific roles for the disease-associated proteins. We demonstrate substantial overlap in the protein interactomes of three transmembrane Batten proteins (CLN3, CLN6, and CLN8), and that their absence leads to synaptic depletion of key partners (i.e., SNAREs and tethers) and altered synaptic SNARE complexing in vivo, demonstrating a novel shared etiology. Frontiers Media S.A. 2022-05-25 /pmc/articles/PMC9174988/ /pubmed/35692423 http://dx.doi.org/10.3389/fnins.2022.834780 Text en Copyright © 2022 Rechtzigel, Meyerink, Leppert, Johnson, Cain, Ferrandino, May, Roux, Brudvig and Weimer. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neuroscience
Rechtzigel, Mitchell J.
Meyerink, Brandon L.
Leppert, Hannah
Johnson, Tyler B.
Cain, Jacob T.
Ferrandino, Gavin
May, Danielle G.
Roux, Kyle J.
Brudvig, Jon J.
Weimer, Jill M.
Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment
title Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment
title_full Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment
title_fullStr Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment
title_full_unstemmed Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment
title_short Transmembrane Batten Disease Proteins Interact With a Shared Network of Vesicle Sorting Proteins, Impacting Their Synaptic Enrichment
title_sort transmembrane batten disease proteins interact with a shared network of vesicle sorting proteins, impacting their synaptic enrichment
topic Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9174988/
https://www.ncbi.nlm.nih.gov/pubmed/35692423
http://dx.doi.org/10.3389/fnins.2022.834780
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