pfam03152: UFD1_N1 (This model is not part of the current CDD release)
Ubiquitin fusion degradation protein UFD1, N-terminal subdomain 1
Post-translational ubiquitin-protein conjugates are recognised for degradation by the ubiquitin fusion degradation (UFD) pathway. Several proteins involved in this pathway have been identified. UFD1 is a 40kD protein that is essential for vegetative cell viability. The human UFD1 gene is expressed at high levels during embryogenesis, especially in the eyes and in the inner ear primordia and is thought to be important in the determination of ectoderm-derived structures, including neural crest cells. In addition, this gene is deleted in the CATCH-22 (cardiac defects, abnormal facies, thymic hypoplasia, cleft palate and hypocalcaemia with deletions on chromosome 22) syndrome. This clinical syndrome is associated with a variety of developmental defects, all characterised by microdeletions on 22q11.2. Two such developmental defects are the DiGeorge syndrome, and the velo-cardio- facial syndrome. Several of the abnormalities associated with these conditions are thought to be due to defective neural crest cell differentiation. UFD1 is composed of an N-terminal conserved region (UT3) and a C-terminal disordered region (UT6) which contains the binding sites for both Npl4 and Cdc48. UFD1 N-terminal domain contains the binding site for ubiquitin and has a similar structure to p97. It is made of two subdomains. This entry corresponds to the first subdomain of UFD1 UT3 which forms a double-psi beta barrel fold.
Jiyao W et al.(2023). "The conserved domain database in 2023.",