A La-related protein modulates 7SK snRNP integrity to suppress P-TEFb-dependent transcriptional elongation and tumorigenesis

Mol Cell. 2008 Mar 14;29(5):588-99. doi: 10.1016/j.molcel.2008.01.003. Epub 2008 Jan 31.

Abstract

The general transcription factor P-TEFb stimulates RNA polymerase II elongation and cotranscriptional processing of pre-mRNA. Contributing to a functional equilibrium important for growth control, a reservoir of P-TEFb is maintained in an inactive snRNP where 7SK snRNA is a central scaffold. Here, we identify PIP7S as a La-related protein stably associated with and required for 7SK snRNP integrity. PIP7S binds and stabilizes nearly all the nuclear 7SK via 3' -UUU-OH, leading to the sequestration and inactivation of P-TEFb. This function requires its La domain and intact C terminus. The latter is frequently deleted in human tumors due to microsatellite instability-associated mutations. Consistent with the tumor suppressor role of a Drosophila homolog of PIP7S, loss of PIP7S function shifts the P-TEFb equilibrium toward the active state, disrupts epithelial differentiation, and causes P-TEFb-dependent malignant transformation. Through PIP7S modulation of P-TEFb, our data thus link a general elongation factor to growth control and tumorigenesis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • 3' Untranslated Regions
  • Animals
  • Autoantigens / genetics
  • Autoantigens / metabolism*
  • Cell Differentiation / physiology
  • Cell Line
  • Cell Transformation, Neoplastic
  • HIV-1 / genetics
  • HIV-1 / metabolism
  • Humans
  • Mammary Glands, Human / cytology
  • Mammary Glands, Human / metabolism
  • Neoplasms* / genetics
  • Neoplasms* / metabolism
  • Positive Transcriptional Elongation Factor B / genetics
  • Positive Transcriptional Elongation Factor B / metabolism*
  • Protein Binding
  • RNA Interference
  • RNA-Binding Proteins / genetics
  • RNA-Binding Proteins / metabolism
  • Ribonucleoproteins / genetics
  • Ribonucleoproteins / metabolism*
  • Ribonucleoproteins, Small Nuclear / genetics
  • Ribonucleoproteins, Small Nuclear / metabolism*
  • SS-B Antigen
  • Transcription Factors
  • Transcription, Genetic*
  • Uridine / chemistry
  • Uridine / metabolism

Substances

  • 3' Untranslated Regions
  • Autoantigens
  • HEXIM1 protein, human
  • RNA-Binding Proteins
  • Ribonucleoproteins
  • Ribonucleoproteins, Small Nuclear
  • Transcription Factors
  • Positive Transcriptional Elongation Factor B
  • Uridine