Girard Mathilde, Goossens Michel
INSERM U654, Bases Moléculaires et Cellulaires des Maladies Génétiques, France.
FEBS Lett. 2006 Mar 6;580(6):1635-41. doi: 10.1016/j.febslet.2006.02.011. Epub 2006 Feb 17.
SRY-related HMG box-containing factor 10 (SOX10) is a transcription factor essential for neural crest development and differentiation, and involved in Waardenburg syndrome type IV and PCWH syndrome. Here we show that the SOX10 protein is modified by sumoylation, a highly dynamic post-translational modification that affects stability, activity and localisation of some specific transcription factors. Three sumoylation consensus sites were found in the SOX10 protein, all of them are functional and modulate SOX10 activity. Sumoylation does not affect SOX10 sub-cellular localisation, but represses its transcriptional activity on two of its target genes, GJB1 and MITF, and modulates its synergy with its cofactors EGR2 and PAX3 on these promoters.
含SRY相关HMG盒的因子10（SOX10）是一种对神经嵴发育和分化至关重要的转录因子，与IV型瓦登伯格综合征和PCWH综合征有关。我们在此表明，SOX10蛋白通过SUMO化修饰，SUMO化是一种高度动态的翻译后修饰，可影响某些特定转录因子的稳定性、活性和定位。在SOX10蛋白中发现了三个SUMO化共有位点，它们均具有功能并调节SOX10活性。SUMO化不影响SOX10的亚细胞定位，但会抑制其对两个靶基因GJB1和MITF的转录活性，并调节其与辅因子EGR2和PAX3在这些启动子上的协同作用。
