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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show...

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Fractionation for Resolution of Soluble and Insoluble Huntingtin Species

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A large scale Huntingtin protein interaction network implicates Rho GTPase signaling pathways in Huntington disease.

Cendrine Tourette¹, Biao Li¹, Russell Bell²

¹Buck Institute for Research on Aging, Novato, California 94945.

The Journal of Biological Chemistry

|January 11, 2014

Summary

Huntington disease (HD) involves CAG expansion in the HTT gene. Researchers found that huntingtin-interacting proteins (HIPs) are linked to pathways affected in HD, suggesting new therapeutic targets for this neurodegenerative disease.

Keywords:

Cell Death Cell Motility Computational Biology Huntington Disease Neurodegeneration Neurodegenerative Diseases Protein-Protein Interactions Proteomics Rho GTPases

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Area of Science:

Neuroscience
Genetics
Molecular Biology

Background:

Huntington disease (HD) is an inherited neurodegenerative disorder.
It is caused by a CAG expansion mutation in the huntingtin (HTT) gene.
The precise molecular mechanisms underlying HD pathogenesis remain incompletely understood.

Purpose of the Study:

To identify and characterize proteins that interact with huntingtin (HTT).
To investigate the functional roles of these HTT-interacting proteins (HIPs) in HD pathology.
To explore the involvement of HTT in cellular processes like membrane dynamics.

Main Methods:

Yeast two-hybrid screening to identify protein-protein interactions.
Bioinformatic analysis of functional annotations for identified protein networks.
Validation of HIPs' roles in mutant HTT toxicity using cellular models.
Confocal microscopy to assess co-localization and cellular dynamics.

Main Results:

A large network of 3235 interactions among 2141 interconnected proteins (HIPs) was identified.
HIPs are significantly enriched in pathways relevant to HD, including mTOR, Rho GTPase signaling, and oxidative stress.
Specific Rho GTPase signaling components (BAIAP2, EZR, PIK3R1, PAK2, RAC1) were validated as modifiers of mutant HTT toxicity.
Huntingtin (Htt) was found to co-localize with BAIAP2 in filopodia, and mutant HTT disrupts filopodial dynamics.

Conclusions:

Huntingtin protein (HTT) plays a direct role in membrane dynamics, cell attachment, and motility.
Dysregulation of Rho GTPase signaling and related pathways contributes to Huntington disease pathogenesis.
Identified HIPs and pathways represent potential therapeutic targets for HD.