Involved in several processes, including negative regulation of protein import into nucleus; protein K69-linked ufmylation; and reticulophagy. Acts upstream of or within response to endoplasmic reticulum stress. Located in cytoplasm and nucleus. Implicated in hypomyelinating leukodystrophy 14.
RGD Description
UFM1 is a ubiquitin-like protein that is conjugated to target proteins by E1-like activating enzyme UBA5 (UBE1DC1; MIM 610552) and E2-like conjugating enzyme UFC1 (MIM 610554) in a manner analogous to ubiquitylation (see UBE2M; MIM 603173) (Komatsu et al., 2004 [PubMed 15071506]).[supplied by OMIM, Dec 2008]