|Other Names||Ubiquitin fusion degradation protein 1 homolog, UB fusion protein 1, UFD1L|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP6626b was selected from the C-term region of human UFD1L. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|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 structures.|
|Cellular Location||Nucleus. Cytoplasm, cytosol|
|Tissue Location||Found in adult heart, skeletal muscle and pancreas, and in fetal liver and kidney|
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UFD1L 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 its gene have been associated with Catch 22 syndrome as well as cardiac and craniofacial defects.
Xie,L., Am. J. Med. Genet. B Neuropsychiatr. Genet. 147B (7), 1076-1079 (2008)Cao,J., Cell Metab. 6 (2), 115-128 (2007)
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