PUBLICATION

Sensory deficit screen identifies nsf mutation that differentially affects SNARE recycling and quality control

Authors
Gao, Y., Khan, Y.A., Mo, W., White, K.I., Perkins, M., Pfuetzner, R.A., Trapani, J.G., Brunger, A.T., Nicolson, T.
ID
ZDB-PUB-230408-44
Date
2023
Source
Cell Reports   42: 112345112345 (Journal)
Registered Authors
Gao, Yan, Mo, Weike, Nicolson, Teresa, Trapani, Josef
Keywords
CP: Neuroscience, NSF, SNARE complex, balance, deafness, hair cell, hearing, ribbon synapse, sensory deficit, zebrafish
MeSH Terms
  • Animals
  • Membrane Fusion*
  • Mutation/genetics
  • N-Ethylmaleimide-Sensitive Proteins/metabolism
  • Quality Control
  • SNARE Proteins*/genetics
  • SNARE Proteins*/metabolism
  • Soluble N-Ethylmaleimide-Sensitive Factor Attachment Proteins/genetics
  • Soluble N-Ethylmaleimide-Sensitive Factor Attachment Proteins/metabolism
  • Zebrafish/genetics
  • Zebrafish/metabolism
PubMed
37027300 Full text @ Cell Rep.
Abstract
The AAA+ NSF complex is responsible for SNARE complex disassembly both before and after membrane fusion. Loss of NSF function results in pronounced developmental and degenerative defects. In a genetic screen for sensory deficits in zebrafish, we identified a mutation in nsf, I209N, that impairs hearing and balance in a dosage-dependent manner without accompanying defects in motility, myelination, and innervation. In vitro experiments demonstrate that while the I209N NSF protein recognizes SNARE complexes, the effects on disassembly are dependent upon the type of SNARE complex and I209N concentration. Higher levels of I209N protein produce a modest decrease in binary (syntaxin-SNAP-25) SNARE complex disassembly and residual ternary (syntaxin-1A-SNAP-25-synaptobrevin-2) disassembly, whereas at lower concentrations binary disassembly activity is strongly reduced and ternary disassembly activity is absent. Our study suggests that the differential effect on disassembly of SNARE complexes leads to selective effects on NSF-mediated membrane trafficking and auditory/vestibular function.
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Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Antibodies
Orthology
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Mapping