Huntingtin Interacting Proteins and Pathological Implications

Huntington's disease (HD) is caused by an expansion of a CAG repeat in the gene that encodes the huntingtin protein (HTT). The exact function of HTT is still not fully understood, and previous studies have mainly focused on identifying proteins that interact with HTT to gain insights into its function. Numerous HTT-interacting proteins have been discovered, shedding light on the functions and structure of HTT. Most of these proteins interact with the N-terminal region of HTT. Among the various HTT-interacting proteins, huntingtin-associated protein 1 (HAP1) and HTT-interacting protein 1 (HIP1) have been extensively studied. Recent research has uncovered differences in the distribution of HAP1 in monkey and human brains compared with mice. This finding suggests that there may be species-specific variations in the regulation and function of HTT-interacting proteins. Understanding these differences could provide crucial insights into the development of HD. In this review, we will focus on the recent advancements in the study of HTT-interacting proteins, with particular attention to the differential distributions of HTT and HAP1 in larger animal models.

Automated summary

Huntington's disease (HD) is caused by an expansion of a CAG repeat in the HTT gene. HTT's exact function is still not fully understood, but previous studies have identified several interacting proteins that shed light on its function and structure. Among these proteins, HAP1 and HIP1 have been extensively studied, and recent research has found differences in their distribution in different animal brains. Understanding these species-specific variations in HTT-interacting proteins could provide crucial insights into HD development.