A novel approach to assess the ubiquitin-fold modifier 1-system in cells

作者：Ishimura Ryosuke; Obata Miki; Kageyama Shun; Daniel Jens; Tanaka Keiji; Komatsu Masaaki^*

来源：FEBS LETTERS, 2017, 591(1): 196-204.

DOI：10.1002/1873-3468.12518

• encephalopathy
• ubiquitin-like modifier
• UFM1

The ubiquitin-fold modifier 1 (UFM1)-system, a ubiquitin-like protein conjugation system, is involved in the development of breast cancer and several hereditary neurological syndromes. However, the molecular mechanisms of UFM1-related pathogenesis remain unclear. Here, we show that in the absence of UFSP2, a deconjugating enzyme for UFM1, ectopic expression of both UFL1 and UFBP1, which serve as the E3-ligase complex for the UFM1-system, dramatically increases UFM1-conjugate formation at the endoplasmic reticulum. Utilizing this system, we were able to attribute disease-related isoforms of UBA5, the E1 enzyme for UFM1, to decreased UFM1-conjugate formation. Our procedure allows the assessment of UFM1-conjugate formation in cells and the identification of UFM1-targets, both of which are needed to clarify the pathophysiological role of the UFM1-system.

• 出版日期2017-1
• 单位河北医科大学