Ubiquitin Fusion Degradation Protein 1 Homolog (UFD1L) (AA 120-241) antibody

Antigen: Ubiquitin Fusion Degradation Protein 1 Homolog (UFD1L)

Synonyms: UFD1, Ufd1, ufd1, MGC68571, zgc:92341, wu:fc55f04, MGC79664, MGC142939

Binding Site: AA 120-241

Clonality: Monoclonal (19)

Host: Mouse

Reactivity: Mouse (Murine)

Conjugate: Un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF)

Catalog no.: ABIN968620

Additional Information

Alternative name: Ufd1L

Immunogen: Mouse Ufd1L

Cross-Reactivity: Human, Rat (Rattus)

Format: Liquid

Isotype: IgG1

Clone: 19

Description: 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, which inhibits 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.

Characteristics: 1. Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2. Please refer to us for technical protocols.
3. Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
4. Source of all serum proteins is from USDA inspected abattoirs located in the United States.

Molecular Weight: 40 kDa

Comments:

Related Products: ABIN968555, ABIN967389

Application Details

Concentration: 250 µg/ml

Purity: Purified

Purification: Purified from tissue culture supernatant or ascites by affinity chromatography.

Buffer: Aqueous buffered solution containing BSA, glycerol.

Preservative: 0.09% Sodium azide.

Storage: Store undiluted at -20° C.

Restrictions: For Research Use only

Images

Western blot analysis of Ufd1L on a mouse macrophage cell lysate. Lane 1: 1:1000, lane 2: 1:2000, lane 3: 1:4000 dilution of mouse anti-Ufd1L antibody.

Immunofluorescence staining of NIH/3T3 cells (Mouse embryo fibroblast cells, ATCC CRL-1658).

anti-Ubiquitin Fusion Degradation Protein 1 Homolog (UFD1L) (AA 120-241) antibody (Image 3)

Publications

Product: Pizzuti, Novelli, Ratti et al.: "UFD1L, a developmentally expressed ubiquitination gene, is deleted in CATCH 22 syndrome." in: Human molecular genetics, Vol. 6, Issue 2, pp. 259-65, 1997 (PubMed).

Yamagishi, Garg, Matsuoka et al.: "A molecular pathway revealing a genetic basis for human cardiac and craniofacial defects." in: Science (New York, N.Y.), Vol. 283, Issue 5405, pp. 1158-61, 1999 (PubMed).

Meyer, Shorter, Seemann et al.: "A complex of mammalian ufd1 and npl4 links the AAA-ATPase, p97, to ubiquitin and nuclear transport pathways." in: The EMBO journal, Vol. 19, Issue 10, pp. 2181-92, 2000 (PubMed).

Nagahama, Suzuki, Hamada et al.: "SVIP is a novel VCP/p97-interacting protein whose expression causes cell vacuolation." in: Molecular biology of the cell, Vol. 14, Issue 1, pp. 262-73, 2003 (PubMed).