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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.<\/jats:p>","DOI":"10.3390\/ijms241713060","type":"journal-article","created":{"date-parts":[[2023,8,22]],"date-time":"2023-08-22T08:58:54Z","timestamp":1692694734000},"page":"13060","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Huntingtin Interacting Proteins and Pathological Implications"],"prefix":"10.3390","volume":"24","author":[{"given":"Li","family":"Liu","sequence":"first","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2978-3128","authenticated-orcid":false,"given":"Huichun","family":"Tong","sequence":"additional","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yize","family":"Sun","sequence":"additional","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xingxing","family":"Chen","sequence":"additional","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3112-9643","authenticated-orcid":false,"given":"Tianqi","family":"Yang","sequence":"additional","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gongke","family":"Zhou","sequence":"additional","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiao-Jiang","family":"Li","sequence":"additional","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1775-6536","authenticated-orcid":false,"given":"Shihua","family":"Li","sequence":"additional","affiliation":[{"name":"Guangdong Key Laboratory of Non-Human Primate Research, Key Laboratory of Central Nervous System Regeneration (Ministry of Education), Guangdong-Hongkong-Macau Institute of CNS Regeneration, Jinan University, Guangzhou 510623, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,8,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1038\/s41582-020-0389-4","article-title":"Huntington disease: New insights into molecular pathogenesis and therapeutic opportunities","volume":"16","author":"Tabrizi","year":"2020","journal-title":"Nat. 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