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Purification of Ubiquitinated p53 Proteins from Mammalian Cells
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Purification of Ubiquitinated p53 Proteins from Mammalian Cells

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Dual regulation of p53 by the ribosome maturation factor SBDS

Qian Hao1,2, Jieqiong Wang3,4,5, Yajie Chen3,4

  • 1Fudan University Shanghai Cancer Center and Institutes of Biomedical Sciences, Fudan University, Shanghai, 200032, China. qhao15@hotmail.com.

Cell Death & Disease
|March 22, 2020
PubMed

Insights

Shwachman-Bodian Diamond syndrome (SBDS) protein promotes cancer by inhibiting p53. Suppressing SBDS halts cancer cell growth and invasion, revealing its dual role in cancer development.

Area of Science:

  • Molecular Biology
  • Cancer Biology
  • Genetics

Background:

  • The Shwachman-Bodian Diamond syndrome (SBDS) gene is linked to rRNA synthesis and ribosome maturation.
  • The specific role of SBDS in cancer development remains largely unknown.

Purpose of the Study:

  • To investigate the function of SBDS in human cancers.
  • To elucidate the molecular mechanisms underlying SBDS's role in tumor progression.

Main Methods:

  • Analysis of SBDS expression and amplification in human cancer samples.
  • Knockdown experiments to assess the impact of SBDS on cancer cell behavior.
  • In vitro and in vivo studies using ectopic SBDS expression.
  • Investigation of the p53-MDM2 pathway interactions.

Main Results:

  • SBDS is frequently overexpressed or amplified in human cancers, correlating with poor prognosis.
  • SBDS knockdown stabilizes and activates p53 via the ribosomal stress-RPL5/RPL11-MDM2 pathway, inhibiting cancer cell proliferation and invasion.
  • Ectopic SBDS in the nucleoplasm suppresses tumor growth.
  • SBDS interacts with p53's transactivation domain, disrupting MDM2-p53 binding and p53 degradation.

Conclusions:

  • SBDS exhibits dual functions in cancer, coordinating ribosome biogenesis and p53 activity.
  • SBDS acts as an oncoprotein by inhibiting p53-mediated tumor suppression.
  • Targeting SBDS may offer a novel therapeutic strategy for cancer treatment.

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