Molecular weight (DA)
33kD
Immunogen
Synthesized peptide derived from part region of human protein
Specificity
UFD1 Polyclonal Antibody detects endogenous levels of protein.
Formulation
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 ELISA 1:5000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
The protein encoded by this gene forms a complex with two other proteins, nuclear protein localization-4 and valosin-containing protein, and this complex is necessary for the degradation of ubiquitinated proteins. In addition, this complex controls the disassembly of the mitotic spindle and the formation of a closed nuclear envelope after mitosis. Mutations in this gene have been associated with Catch 22 syndrome as well as cardiac and craniofacial defects. Alternative splicing results in multiple transcript variants encoding different isoforms. A related pseudogene has been identified on chromosome 18. [provided by RefSeq, Jun 2009],
Function
caution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,disease:UFD1L gene hemizygosity is the cause of some of the catch 22-associated developmental defects whose notable examples are the DiGeorge syndrome (DGS), the velo-cardio-facial syndrome (VCFS) and the Opitz G/BBB syndrome.,function:Essential component of the ubiquitin-dependent proteolytic pathway which degrades ubiquitin fusion proteins. The ternary complex containing UFD1L, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. The NPLOC4-UFD1L-VCP complex regulates spindle disassembly at the end of mitosis and is necessary for the formation of a closed nuclear envelope. It may be involved in the development of some ectoderm-derived stru
Protein name
Ubiquitin fusion degradation protein 1 homolog (UB fusion protein 1)
Fields
>>Protein processing in endoplasmic reticulum
Human protein sequence Database
Q92890
Mouse protein sequence database
P70362
Rat protein sequence database
Q9ES53
Cellular localization
Nucleus . Cytoplasm, cytosol .
Tissue expression
Found in adult heart, skeletal muscle and pancreas, and in fetal liver and kidney.
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