Related F-box proteins control cell death in Caenorhabditis elegans and human lymphoma

Proc Natl Acad Sci U S A. 2013 Mar 5;110(10):3943-8. doi: 10.1073/pnas.1217271110. Epub 2013 Feb 19.

Abstract

Cell death is a common metazoan cell fate, and its inactivation is central to human malignancy. In Caenorhabditis elegans, apoptotic cell death occurs via the activation of the caspase CED-3 following binding of the EGL-1/BH3-only protein to the antiapoptotic CED-9/BCL2 protein. Here we report a major alternative mechanism for caspase activation in vivo involving the F-box protein DRE-1. DRE-1 functions in parallel to EGL-1, requires CED-9 for activity, and binds to CED-9, suggesting that DRE-1 promotes apoptosis by inactivating CED-9. FBXO10, a human protein related to DRE-1, binds BCL2 and promotes its degradation, thereby initiating cell death. Moreover, some human diffuse large B-cell lymphomas have inactivating mutations in FBXO10 or express FBXO10 at low levels. Our results suggest that DRE-1/FBXO10 is a conserved regulator of apoptosis.

Publication types

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

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Apoptosis / physiology*
  • Base Sequence
  • Caenorhabditis elegans / cytology*
  • Caenorhabditis elegans / genetics
  • Caenorhabditis elegans / physiology*
  • Caenorhabditis elegans Proteins / genetics
  • Caenorhabditis elegans Proteins / physiology*
  • Caspases / genetics
  • Caspases / physiology
  • Cell Line, Tumor
  • Enzyme Activation
  • F-Box Proteins / genetics
  • F-Box Proteins / physiology*
  • HEK293 Cells
  • Humans
  • Lymphoma / genetics
  • Lymphoma / pathology*
  • Lymphoma / physiopathology*
  • Lymphoma, Large B-Cell, Diffuse / genetics
  • Lymphoma, Large B-Cell, Diffuse / pathology
  • Lymphoma, Large B-Cell, Diffuse / physiopathology
  • Molecular Sequence Data
  • Mutation, Missense
  • Proto-Oncogene Proteins c-bcl-2 / genetics
  • Proto-Oncogene Proteins c-bcl-2 / physiology
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • RNA, Neoplasm / genetics
  • RNA, Neoplasm / metabolism
  • Repressor Proteins / genetics
  • Repressor Proteins / physiology
  • Sequence Homology, Amino Acid

Substances

  • Caenorhabditis elegans Proteins
  • DRE-1 protein, C elegans
  • EGL-1 protein, C elegans
  • F-Box Proteins
  • Proto-Oncogene Proteins c-bcl-2
  • RNA, Messenger
  • RNA, Neoplasm
  • Repressor Proteins
  • Caspases
  • ced-3 protein, C elegans