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Generation of Native, Untagged Huntingtin Exon1 Monomer and Fibrils Using a SUMO Fusion Strategy
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Calmidazolium Chloride and Its Complex with Serum Albumin Prevent Huntingtin Exon1 Aggregation

Virender Singh1, R N V Krishna Deepak2, Bhaswati Sengupta3

  • 1Biological Sciences and Bioengineering , Indian Institute of Technology Kanpur , Kanpur 208016 , India.

Molecular Pharmaceutics
|July 7, 2018
PubMed

Insights

Calmidazolium chloride inhibits Huntington's disease protein aggregation. A novel formulation using bovine serum albumin nanoparticles enhances this effect, reducing toxic polyglutamine aggregates in cells.

Area of Science:

  • Neuroscience
  • Genetics
  • Biochemistry

Background:

  • Huntington's disease (HD) is a fatal neurodegenerative disorder caused by a CAG repeat expansion in the Huntingtin gene.
  • This expansion leads to a polyglutamine (polyQ) tract in the Huntingtin protein (Httex1), promoting toxic aggregate formation.
  • Current treatments for HD are limited, highlighting the need for novel therapeutic strategies targeting protein aggregation.

Purpose of the Study:

  • To investigate the potential of calmidazolium chloride (CLC) as an inhibitor of Httex1 aggregation.
  • To develop a stabilized formulation of CLC to overcome its adsorption issues and enhance its inhibitory efficacy.
  • To evaluate the therapeutic potential of CLC-loaded nanoparticles in reducing polyQ aggregates in HD models.

Main Methods:

  • In vitro assessment of Httex1 aggregation inhibition by CLC.
  • Development of a CLC-bovine serum albumin (BSA) complex to prevent CLC adsorption.
  • Fabrication and characterization of biocompatible CLC-loaded BSA nanoparticles.
  • Evaluation of nanoparticle efficacy in reducing polyQ aggregates in HD-150Q cells.

Main Results:

  • Calmidazolium chloride demonstrated inhibitory effects on the initial aggregation steps of Httex1.
  • The formation of a BSA-CLC complex successfully prevented CLC adsorption and improved Httex1 aggregation inhibition.
  • BSA nanoparticles loaded with CLC effectively reduced polyglutamine aggregates in HD-150Q cells, indicating therapeutic potential.

Conclusions:

  • Calmidazolium chloride is a promising agent for inhibiting Httex1 aggregation, a key process in Huntington's disease pathogenesis.
  • BSA-based formulations, particularly nanoparticles, can enhance the stability and efficacy of CLC for potential therapeutic applications.
  • CLC-loaded BSA nanoparticles represent a viable strategy for reducing toxic polyQ aggregates in Huntington's disease.

Keywords:
Huntington’s diseaseadsorptioncalmidazolium chloridepolyglutamine aggregationserum albumin

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