The Folding process of Human Profilin-1, a novel protein associated with familial amyotrophic lateral sclerosis

Sci Rep. 2015 Jul 31:5:12332. doi: 10.1038/srep12332.

Abstract

Human profilin-1 is a novel protein associated with a recently discovered form of familial amyotrophic lateral sclerosis. This urges the characterization of possible conformational states, different from the fully folded state, potentially able to initiate self-assembly. Under native conditions, profilin-1 is monomeric and possesses a well-defined secondary and tertiary structure. When incubated at low pH or with high urea concentrations, profilin-1 remains monomeric but populates unfolded states exhibiting larger hydrodynamic radius and disordered structure, as assessed by dynamic light scattering, far-UV circular dichroism and intrinsic fluorescence. Refolding from the urea-unfolded state was studied at equilibrium and in real-time using a stopped-flow apparatus. The results obtained with intrinsic fluorescence and circular dichroism indicate a single phase without significant changes of the corresponding signals before the major refolding transition. However, such a transition is preceded by a burst phase with an observed increase of ANS fluorescence, which indicates the conversion into a transiently populated collapsed state possessing solvent-exposed hydrophobic clusters. Kinetic analysis reveals that such state has a conformational stability comparable to that of the fully unfolded state. To our knowledge, profilin-1 is the first example of an amyloid-related protein where folding occurs in the absence of thermodynamically stable partially folded states.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / metabolism
  • Anilino Naphthalenesulfonates / chemistry
  • Anilino Naphthalenesulfonates / metabolism
  • Circular Dichroism
  • Escherichia coli / genetics
  • Humans
  • Hydrogen-Ion Concentration
  • Kinetics
  • Profilins / chemistry*
  • Profilins / genetics
  • Profilins / metabolism
  • Protein Conformation
  • Protein Folding*
  • Spectrometry, Fluorescence
  • Thermodynamics
  • Urea / chemistry

Substances

  • 8-anilino-1-naphthalenesulfonic acid
  • Anilino Naphthalenesulfonates
  • Profilins
  • Urea