人 TOPORS 抗体对

靶点名称

TOPORS

蛋白名称

TOP1 binding arginine/serine rich protein, E3 ubiquitin ligase

蛋白别名

E3 ubiquitin-protein ligase Topors; RING-type E3 ubiquitin transferase Topors; SUMO1-protein E3 ligase Topors; Topoisomerase I-binding RING finger protein; Topoisomerase I-binding arginine/serine-rich protein; Tumor suppressor p53-binding protein 3; p53-binding protein 3; p53BP3

靶点基因名

TOPORS

基因别名

LUN; TP53BPL

UniProt

Q9NS56

来源物种

Homo sapiens (Human)

物种反应性

Human

背景介绍

TOP1 binding arginine/serine rich protein, E3 ubiquitin ligase (TOPORS) is a dual-function nuclear protein that acts as both an E3 ubiquitin-protein ligase and an E3 SUMO1-protein ligase. It contains a conserved N-terminal RING finger domain that facilitates its interaction with key substrates, including topoisomerase I and the tumor suppressor p53. By promoting the ubiquitin-dependent degradation of p53, TOPORS regulates essential cellular processes such as growth, proliferation, and apoptosis. It also modulates chromatin structure through the sumoylation of proteins like mSin3A. Clinically, TOPORS is of high significance in ophthalmology, as mutations in the TOPORS gene are a direct cause of retinitis pigmentosa 31 (RP31). This hereditary retinal dystrophy is characterized by the progressive degeneration of photoreceptors, leading to night blindness and significant visual field loss. Its high expression in the testis and role in p53 regulation also suggest a function as a probable tumor suppressor.