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Fractionation for Resolution of Soluble and Insoluble Huntingtin Species
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Mitochondrial membranes modify mutant huntingtin aggregation.

Adewale Adegbuyiro1, Faezeh Sedighi1, Pranav Jain1

  • 1The C. Eugene Bennett Department of Chemistry, West Virginia University, 217 Clark Hall, Morgantown, WV 26506, United States.

Biochimica Et Biophysica Acta. Biomembranes
|June 5, 2021
PubMed
Summary
This summary is machine-generated.

Mitochondrial membranes, particularly the inner membrane, significantly influence huntingtin protein aggregation in Huntington's disease (HD). Cardiolipin content modulates this effect, impacting disease pathology.

Keywords:
Amyloid fibrilsCardiolipinHuntington's diseaseMitochondriaOligomersPolyglutamine

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Area of Science:

  • Neuroscience
  • Biochemistry
  • Cell Biology

Background:

  • Huntington's disease (HD) is a neurodegenerative disorder linked to expanded polyglutamine (polyQ) in huntingtin (htt) protein.
  • Mutant htt (mhtt) aggregates and causes mitochondrial dysfunction, a hallmark of HD.
  • Mitochondrial lipids, like cardiolipin (CL), are crucial for mitochondrial function and may interact with htt.

Purpose of the Study:

  • To investigate how mitochondrial membranes affect huntingtin protein aggregation.
  • To explore the role of cardiolipin in modulating mhtt aggregation.

Main Methods:

  • Utilized outer and inner mitochondrial membrane mimics (OMM and IMM) to study mhtt aggregation.
  • Employed atomic force microscopy (AFM) to visualize aggregation patterns.
  • Investigated mhtt aggregation in lipid systems with varying cardiolipin concentrations.

Main Results:

  • Both OMM and IMM reduced mhtt fibrillization, with IMM showing a greater inhibitory effect.
  • Low cardiolipin (<5%) promoted mhtt fibrillization, while higher concentrations inhibited it.
  • AFM revealed distinct mhtt aggregation morphologies on OMM versus IMM surfaces.

Conclusions:

  • Mitochondrial membranes play a significant role in regulating huntingtin protein aggregation.
  • Cardiolipin content is a critical factor influencing mhtt aggregation dynamics.
  • These findings offer insights into HD pathogenesis and potential therapeutic targets.