Ufd1L Pure 19/Ufd1L 50ug
货号:
611642 基本售价:
3726.0 元 规格:
50ug
产品信息
概述货号 | 611642 |
描述 | Microdeletions of the chromosomal region 22q11.2 are associated with defects in cardiac and craniofacial development that are referred to as the CATCH 22 phenotype and include DiGeorge syndrome, the velo-cardio-facial syndrome, and the Opitz GBBB syndrome. Deleted in CATCH 22 patients, UFD1L encodes the human homolog of the yeast ubiquitin fusion degradation 1 protein (UFD1p), an essential component of the ubiquitin-dependent proteolytic pathway. The murine homolog (Ufd1l) is expressed in the eyes and in the inner ear primordia during embryogenesis. In rat liver cytosol, Ufd1l binds to the nuclear transport protein Npl4 and this binary complex competes with p47 for binding to p97, whichinhibits Golgi membrane fusion. The tertiary complex Ufd1l/Npl4/p97 may also be involved in mitotic ubiquitin-dependent processes. Both Ufd1l and Npl4 are found in the nucleus, while p97 is found in the cytoplasm and nucleus. In humans, Ufd1L is primarily expressed in adult heart, placenta, skeletal muscle, and pancreas, and fetal liver and kidney. Thus, Ufd1L is thought to be involved in cell functions that are critical for normal cell development in the cardiac and craniofacial regions. |
同种亚型 | IgG1 |
反应种属 | Mouse (QC Testing) Human, Rat (Tested in Development) |
来源宿主 | Mouse |
使用方法 | WB, IF |
性能供应商 | BD Pharmingen |
免疫原 | Mouse Ufd1L aa. 120-241 |
存放说明 | The monoclonal antibody was purified from tissue culture supernatant or ascites by affinity chromatography.Store undiluted at -20°C.
|
存储溶液 | Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide. |
浓度 | 250 µg/ml |