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Disclosing Pathogenic Variant Effects on the Structural Dynamics of the VAPB MSP Domain Causing Familial ALS.

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Pathogenic variants in the VAMP-associated protein B (VAPB) MSP domain disrupt its structural dynamics, impacting cellular functions and potentially causing amyotrophic lateral sclerosis (ALS8). Understanding these changes is key to developing new ALS therapies.

Keywords:
amyotrophic lateral sclerosis 8 (ALS8)major sperm (MSP) domainmolecular dynamics (MD) simulationvesicle-associated membrane protein (VAMP)-associated protein B (VAPB)

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Biochemistry

Background:

  • Vesicle-associated membrane protein (VAMP)-associated protein B (VAPB) is crucial for ER functions, including organelle tethering and lipid transfer.
  • Pathogenic variations in the VAPB MSP domain are linked to amyotrophic lateral sclerosis type 8 (ALS8).
  • The precise molecular mechanisms underlying ALS8 pathogenesis due to VAPB mutations remain unclear.

Purpose of the Study:

  • To investigate the structural and dynamic consequences of pathogenic VAPB MSP domain variants using molecular dynamics (MD) simulations.
  • To elucidate the molecular pathophysiology of ALS8 linked to VAPB mutations.

Main Methods:

  • Molecular dynamics (MD) simulations were employed to analyze the VAPB MSP domain.
  • Secondary structure analysis was performed to assess changes in protein organization.

Main Results:

  • Pathogenic VAPB variants significantly altered protein fluctuations and conformational dynamics.
  • Mutations led to increased alpha-helix content and decreased beta-sheet formation in the VAPB MSP domain.
  • These structural changes may impair VAPB's function in organelle tethering and protein interactions.

Conclusions:

  • Pathogenic VAPB variants induce detrimental structural and dynamic alterations in the MSP domain.
  • These findings provide insights into ALS8 pathophysiology and suggest therapeutic strategies targeting VAPB structure.
  • Further in vivo and in vitro studies are warranted to validate these findings and develop treatments.