Increased protein levels of heterogeneous nuclear ribonucleoprotein A2/B1 in fetal Down syndrome brains

  • S. H. Kim
  • M. Dierssen
  • J. C. Ferreres
  • M. Fountoulakis
  • G. Lubec


Heterogeneous nuclear ribonucleoproteins (hnRNPs) are predominantly nuclear RNA-binding proteins that form complexes with RNA polymerase II transcripts. These proteins play pivotal roles in transcription, pre-mRNA processing in the nucleus, cytoplasmic mRNA translation and its turnover. In addition, hnRNPs have been shown to be essential for embryonic development of Drosophila. Here we studied the protein levels of hnRNPs (A2/B1, H and H’) in fetal brain with Down syndrome (DS; n = 5) compared to controls (n = 7). We used two-dimensional (2-D) gel electrophoresis, matrix-assisted laser desorption ionization mass spectroscopy (MALDI-MS) and specific software for quantification. hnRNP A2/B1 was significantly increased in fetal DS brain (13.52 ± 4.50) compared to controls (9.16 ± 1.35), but both hnRNP H and H’ were unchanged. Increased hnRNP A2/B1 in fetal DS brain may represent high activity of RNA processing such as RNA trafficking and telomere protection, and/or it could contribute to abnormal development of DS brains. Furthermore, comparable expression of hnRNP H and H’ suggest a specific upregulation of hnRNP A2/B.


Down Syndrome Fetal Brain Heterogeneous Nuclear Ribonucleoprotein hnRNP Protein Colloidal Coomassie Blue 
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© Springer-Verlag/Wien 2001

Authors and Affiliations

  • S. H. Kim
    • 1
  • M. Dierssen
    • 2
  • J. C. Ferreres
    • 3
  • M. Fountoulakis
    • 4
  • G. Lubec
    • 1
  1. 1.Department of PediatricsUniversity of ViennaAustria
  2. 2.Medical and Molecular Genetics Center-IROHospital Duran i ReynalsBarcelonaSpain
  3. 3.Department of Pathology UDIAT-CDCorporacis Sanit’ria Parc TauliBarcelonaSpain
  4. 4.F. Hoffmann - La RocheBaselSwitzerland

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