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RpA1 ameliorates symptoms of mutant ataxin-1 knock-in mice and enhances DNA damage repair.

Human molecular genetics - 15 Oct 2016

Taniguchi Juliana Bosso, Kondo Kanoh, Fujita Kyota, Chen Xigui, Homma Hidenori, Sudo Takeaki, Mao Ying, Watase Kei, Tanaka Toshihiro, Tagawa Kazuhiko, Tamura Takuya, Muramatsu Shin-Ichi, Okazawa Hitoshi

Abstract excerpt

DNA damage and repair is a critical domain of many neurodegenerative diseases. In this study, we focused on RpA1, a candidate key molecule in polyQ disease pathologies, and tested the therapeutic effect of adeno-associated virus (AAV) vector expressing RpA1 on mutant Ataxin-1 knock-in (Atxn1-KI) mice. We found significant effects on motor functions, normalized DNA damage markers (γH2AX and 53BP1), and improved...

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PMID: 28173122
DOI: 10.1093/hmg/ddw272

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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