Search Thermo Fisher Scientific
Search Thermo Fisher Scientific
Antibody detects endogenous levels of total UFD1L.
Ubiquitin-mediated proteolysis requires the transfer of ubiquitin (Ub) to lysine groups on selected cellular proteins, which then potentiates the proteolytic degradation of these protein conjugates by the 26S proteasome. Ub-fusions are cleaved by Ub-specific processing proteases (UBps) or alternatively by the Ub-fusion degradation (UFD) pathway. The UBP pathway targets the C-terminal glycine residue on Ub that is involved in the formation of Ub-conjugates, while UFD proteins preferentially cleave Ub-conjugated proteins that contain an amino acid substitution at this glycine residue. The UFD1 protein was originally characterized in the yeast S. cerevisiae and subsequently, the human homolog UFD1 or UFD1L was identified. In vitro, UFD1 attenuates the degradation of Ub-fusions, which have a proline or valine residue substituted at the Gly76 moiety, by the selective multiubiquitination of the Ub chain of the Ub-conjugate. Mutations within the UFD1 gene are implicated in the development of CATCH22 syndrome, which is characterized by cardiac defects, cleft palate and hypocalcemia, suggesting that this proteolytic pathway may be involved in the progression of these developmental defects.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: UB fusion protein 1; UB fusion protein 1 {ECO:0000250|UniProtKB:Q92890}; Ubiquitin fusion degradation protein 1; Ubiquitin fusion degradation protein 1 homolog; ubiquitin fusion degradation protein 1 homolog {ECO:0000250|UniProtKB:Q92890}; Ubiquitin recognition factor in ER-associated degradation protein 1; ufd1l {ECO:0000312|RGD:619822}
Gene Aliases: UFD1; UFD1L
UniProt ID: (Human) Q92890, (Rat) Q9ES53, (Mouse) P70362
Entrez Gene ID: (Human) 7353, (Rat) 84478, (Mouse) 22230
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
Learn moreGet expert recommendations for common problems or connect directly with an on staff expert for technical assistance related to applications, equipment and general product use.
Contact tech support