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Related Experiment Video

Updated: Oct 24, 2025

Efficient and Scalable Production of Full-length Human Huntingtin Variants in Mammalian Cells using a Transient Expression System
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HAP40 protein levels are huntingtin-dependent and decrease in Huntington disease.

Bin Huang1, Manuel Seefelder1, Eva Buck2

  • 1Department of Gene Therapy, Ulm University, 89081 Ulm, Germany.

Neurobiology of Disease
|August 14, 2021
PubMed
Summary
This summary is machine-generated.

Huntingtin-associated protein 40 (HAP40) levels depend on huntingtin (HTT). Reduced HAP40 in Huntington Disease (HD) suggests HAP40 may be crucial for HD pathophysiology.

Keywords:
HAP40HuntingtinHuntington diseaseObligate partnerProtein stability

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Huntingtin-associated protein 40 (HAP40) is a key interactor of huntingtin (HTT).
  • The interaction between HAP40 and HTT is evolutionarily conserved.
  • HAP40 is stabilized by binding to HTT, increasing its half-life.

Purpose of the Study:

  • To investigate the relationship between HAP40 and HTT protein levels.
  • To determine if HAP40 levels are altered in Huntington's Disease (HD).
  • To explore the potential role of HAP40 in HD pathophysiology.

Main Methods:

  • Assessed HAP40 protein levels in relation to HTT.
  • Quantified HAP40 and soluble HTT in HD patient-derived cells (fibroblasts, lymphoblasts).
  • Examined HAP40 levels in brain tissue from a full-length HTT mouse model of HD.

Main Results:

  • HAP40 protein levels are directly dependent on the presence of HTT (both normal and mutant).
  • HAP40 is significantly stabilized when bound to HTT, with a 5-fold increase in half-life.
  • Reduced cellular HAP40 levels were observed in HD patient cells and in the HD mouse model, correlating with decreased soluble HTT.

Conclusions:

  • HAP40 is an obligate interaction partner of HTT.
  • Decreased HAP40 levels in HD models suggest a potential contribution to HD pathophysiology.
  • Further research is warranted to investigate HAP40 loss-of-function in HD.