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Transmembrane Batten disease proteins interact with a shared network of vesicle sorting proteins, impacting synaptic composition and function

ABSTRACT: 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.

INSTRUMENT(S): Q Exactive Plus

ORGANISM(S): Mus Musculus (ncbitaxon:10090)

SUBMITTER: Jill M. Weimer

PROVIDER: MSV000089081 | MassIVE | Sat Mar 19 00:26:00 GMT 2022

SECONDARY ACCESSION(S): PXD032624

REPOSITORIES: MassIVE

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

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