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Research interests
Huntington’s disease (HD) is hallmarked by the accumulation and aggregation of mutant huntingtin protein (mHTT) fragments. Our goal is to improve recognition and degradation of mHTT by the ubiquitin-proteasome system.
An increasing number of neurodegenerative disorders is characterized by the accumulation and aggregation of particular proteins. Here, aggregates are often formed by proteolytic fragments and not by full-length proteins, as is the case with Huntington's and Alzheimer's disease. This suggests that they share a common mechanism in the cellular inability to remove certain peptides which start forming toxic oligomeric structures that may kill the cell unless stored into protective aggresomes. Peptidases may play a crucial role in the cellular attempt to degrade the monomeric fragments once released from the original proteins, and may even play in concert with heat shock proteins to dismantle oligomeric structures into fragments that can subsequently be degraded.
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Collaborations and top research areas from the last five years
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Dynamic recruitment of ubiquitin to mutant huntingtin inclusion bodies
Juenemann, K., Jansen, A. H. P., van Riel, L., Merkx, R., Mulder, M. P. C., An, H., Statsyuk, A., Kirstein, J., Ovaa, H. & Reits, E. A., 2018, In: Scientific reports. 8, 1, p. 1405Research output: Contribution to journal › Article › Academic › peer-review
Open AccessFile30 Downloads (Pure) -
Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific
Jansen, A. H. P., van Hal, M., Op den Kelder, I. C., Meier, R. T., de Ruiter, A.-A., Schut, M. H., Smith, D. L., Grit, C., Brouwer, N., Kamphuis, W., Boddeke, H. W. G. M., den Dunnen, W. F. A., van Roon, W. M. C., Bates, G. P., Hol, E. M. & Reits, E. A., 2017, In: Glia. 65, 1, p. 50-61Research output: Contribution to journal › Article › Academic › peer-review
Open AccessFile16 Downloads (Pure) -
Tripeptidyl Peptidase II Mediates Levels of Nuclear Phosphorylated ERK1 and ERK2
Wiemhoefer, A., Stargardt, A., van der Linden, W. A., Renner, M. C., van Kesteren, R. E., Stap, J., Raspe, M. A., Tomkinson, B., Kessels, H. W., Ovaa, H., Overkleeft, H. S., Florea, B. & Reits, E. A., 2015, In: Molecular & cellular proteomics. 14, 8, p. 2177-2193Research output: Contribution to journal › Article › Academic › peer-review
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Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies
Schipper-Krom, S., Juenemann, K., Jansen, A. H., Wiemhoefer, A., van den Nieuwendijk, R., Smith, D. L., Hink, M. A., Bates, G. P., Overkleeft, H., Ovaa, H. & Reits, E., 2014, In: FEBS letters. 588, 1, p. 151-159Research output: Contribution to journal › Article › Academic › peer-review
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Expanded Polyglutamine-containing N-terminal Huntingtin Fragments Are Entirely Degraded by Mammalian Proteasomes
Juenemann, K., Schipper-Krom, S., Wiemhoefer, A., Kloss, A., Sanz Sanz, A. & Reits, E. A. J., 2013, In: Journal of biological chemistry. 288, 38, p. 27068-27084Research output: Contribution to journal › Article › Academic › peer-review