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. 

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

JOURNAL=Frontiers in Neuroscience

VOLUME=16

YEAR=2022

URL=https://www.frontiersin.org/journals/neuroscience/articles/10.3389/fnins.2022.834780

DOI=10.3389/fnins.2022.834780

ISSN=1662-453X

ABSTRACT=<p>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 <italic>in vivo</italic>, demonstrating a novel shared etiology.</p>