Coordinated and widespread expression of gamma-secretase in vivo: evidence for size and molecular heterogeneity

Neurobiol Dis. 2004 Nov;17(2):260-72. doi: 10.1016/j.nbd.2004.08.002.

Abstract

Gamma-secretase is a high molecular weight protein complex composed of four subunits, namely, presenilin (PS; 1 or 2), nicastrin, anterior pharynx defective-1 (Aph-1; A or B), and presenilin enhancer-2 (Pen-2), and is responsible for the cleavage of a number of type-1 transmembrane proteins. A fundamental question is whether different gamma-secretase complexes exist in vivo. We demonstrate here by in situ hybridization and by Northern and Western blotting that the gamma-secretase components are widely distributed in all tissues investigated. The expression of the different subunits seems tightly coregulated. However, some variation in the expression of the Aph-1 proteins is observed, Aph-1A being more general and abundantly distributed than Aph-1B. The previously uncharacterized rodent-specific Aph-1C mRNA is highly expressed in the kidney and testis but not in brain or other tissues, indicating some tissue specificity for the Aph-1 component of the gamma-secretase complex. Blue-native electrophoresis revealed size heterogeneity of the mature gamma-secretase complex in various tissues. Using co-immunoprecipitations and blue-native electrophoresis at endogenous protein levels, we find evidence that several independent gamma-secretase complexes can coexist in the same cell type. In conclusion, our results suggest that gamma-secretase is a heterogeneous family of protein complexes widely expressed in the adult organism.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Amyloid Precursor Protein Secretases
  • Animals
  • Aspartic Acid Endopeptidases
  • Blotting, Western
  • Cell Line
  • Chromosome Segregation
  • Endopeptidases / chemistry
  • Endopeptidases / metabolism*
  • HeLa Cells
  • Humans
  • Isoenzymes / metabolism
  • Membrane Proteins / genetics
  • Membrane Proteins / metabolism
  • Mice
  • Mice, Knockout
  • Molecular Sequence Data
  • Molecular Weight
  • Peptide Hydrolases
  • Presenilin-1
  • Presenilin-2
  • Protein Isoforms / metabolism
  • RNA, Messenger / metabolism
  • Tissue Distribution
  • Transfection

Substances

  • Isoenzymes
  • Membrane Proteins
  • PSEN1 protein, human
  • PSEN2 protein, human
  • PSENEN protein, human
  • Presenilin-1
  • Presenilin-2
  • Protein Isoforms
  • RNA, Messenger
  • APH1A protein, human
  • Amyloid Precursor Protein Secretases
  • Endopeptidases
  • Peptide Hydrolases
  • Aspartic Acid Endopeptidases
  • BACE1 protein, human
  • Bace1 protein, mouse