Analysis of intracellular distribution and apoptosis involvement of the Ufd1l gene product by over-expression studies

Francesca Amati, Ivano Condò, Emanuela Conti, Federica Sangiuolo, Bruno Dallapiccola, Roberto Testi, Giuseppe Novelli

Research output: Contribution to journalArticle


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.

Original languageEnglish
Pages (from-to)263-267
Number of pages5
JournalCell Biochemistry and Function
Issue number3
Publication statusPublished - Sep 2003



  • Apoptosis
  • Cellular localization
  • Proteasome
  • Ubiquitin

ASJC Scopus subject areas

  • Clinical Biochemistry
  • Cell Biology

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