title: Analysis of intracellular distribution and apoptosis involvement of the Ufd1l gene product by over-expression studies.
abstract: UFD1L is the human homologue of the yeast ubiquitin fusion degradation 1 (Ufd1) gene and maps on chromosome 22q11.2 in the typically deleted region (TDR) for DiGeorge/velocardiofacial syndromes (DGS/VCFS). In yeast, Ufd1 protein is involved in a degradation pathway for ubiquitin fused products (UFD pathway). Several studies have demonstrated that Ufd1 is a component of the Cdc48-Ufd1-Npl4 multiprotein complex which is active in the recognition of several polyubiquitin-tagged proteins and facilitates their presentation to the 26S proteasome for protein degradation or even more specific processing. The multiprotein complex Cdc48-Ufd-Npl4 is also active in mammalian cells. The biochemical role of UFD1L protein in human cells is unknown, even though the interaction between UFD1L and NPL4 proteins has been maintained. In order to clarify this issue, we examined the intracellular distribution of the protein in different mammalian cells and studied its involvement in the Fas and ceramide factors-mediated apoptotic pathways. We established that in mammalian cells, Ufd1l is localized around the nucleus and that it does not interfere with Fas-and ceramide-mediated apoptosis.
authors.affiliation: Dipartimento di Biopatologia e Diagnostica per Immagini, Università di Roma Tor Vergata, Rome, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Amati
authors.forename: Ivano
authors.initials: I
authors.lastname: Condò
authors.forename: Emanuela
authors.initials: E
authors.lastname: Conti
authors.forename: Federica
authors.initials: F
authors.lastname: Sangiuolo
authors.forename: Bruno
authors.initials: B
authors.lastname: Dallapiccola
authors.forename: Roberto
authors.initials: R
authors.lastname: Testi
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Novelli
mesh_terms: D033942:Adaptor Proteins, Vesicular Transport; D000818:Animals; D000911:Antibodies, Monoclonal / Q000494:pharmacology; D058846:Antibodies, Monoclonal, Murine-Derived; D017209:Apoptosis / Q000187:drug effects / Q000502:physiology*; D019556:COS Cells; D002522:Chlorocebus aethiops; D003001:Cloning, Molecular; D005786:Gene Expression Regulation / Q000235:genetics; D005822:Genetic Vectors / Q000235:genetics; D049452:Green Fluorescent Proteins; D006801:Humans; D051034:Inhibitor of Apoptosis Proteins; D036341:Intercellular Signaling Peptides and Proteins; D047908:Intracellular Signaling Peptides and Proteins; D042541:Intracellular Space / Q000378:metabolism; D008164:Luminescent Proteins / Q000235:genetics / Q000378:metabolism; D051379:Mice; D008856:Microscopy, Fluorescence / Q000379:methods; D041681:NIH 3T3 Cells; D011506:Proteins / Q000235:genetics / Q000378:metabolism / Q000502:physiology*; D011993:Recombinant Fusion Proteins / Q000235:genetics / Q000378:metabolism; D013110:Sphingosine / Q000031:analogs & derivatives* / Q000494:pharmacology; D014162:Transfection / Q000379:methods; D014407:Tumor Cells, Cultured; D014764:Viral Proteins / Q000494:pharmacology
chemical_list: D033942:Adaptor Proteins, Vesicular Transport; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D051034:Inhibitor of Apoptosis Proteins; D036341:Intercellular Signaling Peptides and Proteins; D047908:Intracellular Signaling Peptides and Proteins; D008164:Luminescent Proteins; C064769:N-acetylsphingosine; D011506:Proteins; D011993:Recombinant Fusion Proteins; C491497:UFD1 protein, human; C107396:Ufd1 protein, mouse; D014764:Viral Proteins; C411023:anti-Fas monoclonal antibody; C086430:inhibitor of apoptosis, Nucleopolyhedrovirus; D049452:Green Fluorescent Proteins; D013110:Sphingosine