CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination. (Articolo in rivista)

Type

• Articolo in rivista (Classe)
• Prodotto della ricerca (Classe)

Label

• CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination. (Articolo in rivista) (literal)

Anno

• 2011-01-01T00:00:00+01:00 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi

• 10.4161/cc.10.21.17905 (literal)

Alternative label

• Latini P, Frontini M, Caputo M, Gregan J, Cipak L, Filippi S, Kumar V, Velez-Cruz R, Stefanini M and Proietti-De-Santis L. (2011)
  CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination.
  in Cell cycle (Georget. Tex.)
  (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori

• Latini P, Frontini M, Caputo M, Gregan J, Cipak L, Filippi S, Kumar V, Velez-Cruz R, Stefanini M and Proietti-De-Santis L. (literal)

Pagina inizio

• 3719 (literal)

Pagina fine

• 3730 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#url

• http://www.landesbioscience.com/journals/cc/article/17905/ (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume

• 10 (literal)

Rivista

• Cell cycle (Rivista)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroFascicolo

• 21 (literal)

Note

• PubMe (literal)
• ISI Web of Science (WOS) (literal)
• Scopu (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni

• Unit of Molecular Genetics of Aging, DeB; University of tuscia; Viterbo, Italy; Clinical Sciences Centre; Medical research Council Faculty of Medicine Imperial College London; Hammersmith Hospital Campus; London, United Kingdom; Max F. Perutz Laboratories; University of Vienna; Vienna, Austria; Laboratory of Cancer Genetics; Cancer research Institute; Slovak Academy of Sciences; Bratislava, Slovak republic; Institut de Génétique et de Biologie Moléculaire et Cellulaire; CNrS/INSerM; Strasbourg, France; 6Laboratorio Genetica Umana; Instituto Genetica Molecolare; Pavia, Italy (literal)

Titolo

• CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination. (literal)

Abstract

• Mutations in Cockayne syndrome (CS) A and B genes (CSA and CSB) result in a rare genetic disease that affects the development and homeostasis of a wide range of tissues and organs. We previously correlated the degenerative phenotype of patients to the enhanced apoptotic response, exhibited by CS cells, which is associated with the exceptional induction of p53 protein, upon a variety of stress stimuli. Here we showed that the elevated and persistent levels of p53 displayed by CS cells are due to the insufficient ubiquitination of the p53 protein. We further demonstrated that CSA and CSB proteins associate in a unique complex with p53 and Mdm2; this interaction greatly stimulates the ubiquitination of p53 in an Mdm2-dependent manner. Tandem affinity purification and immunoprecipitations combined with mass spectrometry studies indicate that CSA and CSB associate within a Cullin Ring Ubiquitin Ligase complex responsible, under certain circumstances, for p53 ubiquitination. This study identifies CSA and CSB as the key elements of a regulatory mechanism that equilibrate beneficial and detrimental effects of p53 activity upon cellular stress. The deregulation of p53, in absence of either of the CS proteins, can potentially explain the early onset degeneration of tissues and organs observed in CS patients. (literal)

Prodotto di

• Institute of molecular genetics (IGM) (Istituto)
• Malattie genetiche dovute a difetti nella riparazione del DNA che predispongono ai tumori. Analisi genetica funzionale dei fattori importanti per l'integrità del genoma. (ME.P05.005.001) (Modulo)

Autore CNR

• MIRIA STEFANINI (Unità di personale interno)

Insieme di parole chiave

• Keywords of "CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination." (Insieme di parole chiave)

Incoming links:

Prodotto

• Institute of molecular genetics (IGM) (Istituto)
• Malattie genetiche dovute a difetti nella riparazione del DNA che predispongono ai tumori. Analisi genetica funzionale dei fattori importanti per l'integrità del genoma. (ME.P05.005.001) (Modulo)

Autore CNR di

• MIRIA STEFANINI (Unità di personale interno)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#rivistaDi

• Cell cycle (Rivista)

Insieme di parole chiave di

• Keywords of "CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination." (Insieme di parole chiave)