DNAJB6, a Key Factor in Neuronal Sensitivity to Amyloidogenesis

Arun Thiruvalluvan; Eduardo P. de Mattos; Jeanette F. Brunsting; Rob Bakels; Despina Serlidaki; Lara Barazzuol; Paola Conforti; Azra Fatima; Seda Koyuncu; Elena Cattaneo; David Vilchez; Steven Bergink; Erik H. W. G. Boddeke; Sjef Copray; Harm H. Kampinga

doi:10.1016/j.molcel.2020.02.022

DNAJB6, a Key Factor in Neuronal Sensitivity to Amyloidogenesis

Arun Thiruvalluvan, Eduardo P. de Mattos, Jeanette F. Brunsting, Rob Bakels, Despina Serlidaki, Lara Barazzuol, Paola Conforti, Azra Fatima, Seda Koyuncu, Elena Cattaneo, David Vilchez, Steven Bergink, Erik H. W. G. Boddeke, Sjef Copray, Harm H. Kampinga^*

^*Bijbehorende auteur voor dit werk

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Thiruvalluvan et al. show that, upon differentiation, the cellular chaperone network is rewired. This includes a downregulation of the Hsp70 co-chaperone DNAJB6. High levels of DNAJB6 keep patient-derived neuronal progenitors resistant to the formation of amyloids. The sole knockdown of DNAJB6 in progenitors induces spontaneous aggregation of endogenously expressed polyglutamine proteins. Inversely, low levels of DNAJB6 in neurons render them hypersensitive to amyloid formation, which can be restored by re-elevating DNAJB6 expression levels.

Originele taal-2	English
Pagina's (van-tot)	346-+
Aantal pagina's	22
Tijdschrift	Molecular Cell
Volume	78
Nummer van het tijdschrift	2
Vroegere onlinedatum	2020
DOI's	https://doi.org/10.1016/j.molcel.2020.02.022
Status	Published - 16-apr.-2020

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10.1016/j.molcel.2020.02.022

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Thiruvalluvan, A., de Mattos, E. P., Brunsting, J. F., Bakels, R., Serlidaki, D., Barazzuol, L., Conforti, P., Fatima, A., Koyuncu, S., Cattaneo, E., Vilchez, D., Bergink, S., Boddeke, E. H. W. G., Copray, S., & Kampinga, H. H. (2020). DNAJB6, a Key Factor in Neuronal Sensitivity to Amyloidogenesis. Molecular Cell, 78(2), 346-+. https://doi.org/10.1016/j.molcel.2020.02.022

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title = "DNAJB6, a Key Factor in Neuronal Sensitivity to Amyloidogenesis",

abstract = "Thiruvalluvan et al. show that, upon differentiation, the cellular chaperone network is rewired. This includes a downregulation of the Hsp70 co-chaperone DNAJB6. High levels of DNAJB6 keep patient-derived neuronal progenitors resistant to the formation of amyloids. The sole knockdown of DNAJB6 in progenitors induces spontaneous aggregation of endogenously expressed polyglutamine proteins. Inversely, low levels of DNAJB6 in neurons render them hypersensitive to amyloid formation, which can be restored by re-elevating DNAJB6 expression levels.",

keywords = "HEAT-SHOCK PROTEINS, POLYGLUTAMINE AGGREGATION, HUNTINGTONS-DISEASE, ASYMMETRIC INHERITANCE, MOLECULAR CHAPERONES, ANDROGEN RECEPTOR, DAMAGED PROTEINS, RICH REGION, CELL LINES, MUTATIONS",

author = "Arun Thiruvalluvan and {de Mattos}, {Eduardo P.} and Brunsting, {Jeanette F.} and Rob Bakels and Despina Serlidaki and Lara Barazzuol and Paola Conforti and Azra Fatima and Seda Koyuncu and Elena Cattaneo and David Vilchez and Steven Bergink and Boddeke, {Erik H. W. G.} and Sjef Copray and Kampinga, {Harm H.}",

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AU - Brunsting, Jeanette F.

AU - Bakels, Rob

AU - Serlidaki, Despina

AU - Barazzuol, Lara

AU - Conforti, Paola

AU - Fatima, Azra

AU - Koyuncu, Seda

AU - Cattaneo, Elena

AU - Vilchez, David

AU - Bergink, Steven

AU - Boddeke, Erik H. W. G.

AU - Copray, Sjef

AU - Kampinga, Harm H.

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AB - Thiruvalluvan et al. show that, upon differentiation, the cellular chaperone network is rewired. This includes a downregulation of the Hsp70 co-chaperone DNAJB6. High levels of DNAJB6 keep patient-derived neuronal progenitors resistant to the formation of amyloids. The sole knockdown of DNAJB6 in progenitors induces spontaneous aggregation of endogenously expressed polyglutamine proteins. Inversely, low levels of DNAJB6 in neurons render them hypersensitive to amyloid formation, which can be restored by re-elevating DNAJB6 expression levels.

KW - HEAT-SHOCK PROTEINS

KW - POLYGLUTAMINE AGGREGATION

KW - HUNTINGTONS-DISEASE

KW - ASYMMETRIC INHERITANCE

KW - MOLECULAR CHAPERONES

KW - ANDROGEN RECEPTOR

KW - DAMAGED PROTEINS

KW - RICH REGION

KW - CELL LINES

KW - MUTATIONS

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DO - 10.1016/j.molcel.2020.02.022

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SP - 346-+

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JF - Molecular Cell

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