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. 2019 Jul 18;7:103. doi: 10.1186/s40478-019-0758-7

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© The Author(s). 2019

Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Fig. 1 — Schematic representation of the domain architecture of the human Ubiquilin-2 gene. UBQLN2 gene is in the chromosome Xp11.21 and have only one coding exon. UBQLN2 possess a ubiquitin-like domain (UBL) domain on the N-terminal that interacts with the proteasome and a ubiquitin-associated domain (UBA) on the C-terminal required for the UPS activity. UBQLN2 gene also arbors four stress-induced protein 1 (STI-1)-like motif and a proline-rich repeat domain containing 12 PXX repeats. UBL: Ubiquitin-like domain; STI-1: Stress-induced protein 1; UBA: Ubiquitin-associated domain; HSP70: Heat-shock protein 70; Ub: Ubiquitin