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Misfolded proteins with abnormal polyglutamine (polyQ) expansion cause neurodegenerative disorders, including Huntington's disease. Recently, it was found that polyQ aggregates accumulate as a result of vaccinia-related kinase 2 (VRK2)-mediated degradation of TCP-1 ring complex (TRiC)/chaperonin-containing TCP-1 (CCT), which has an essential role in the prevention of polyQ protein aggregation and cytotoxicity. The levels of VRK2 are known to be much higher in actively proliferating cells but are maintained at a low level in the brain via an unknown mechanism. Here, we found that basal levels of neuronal cell-specific VRK2 mRNA are maintained by post-transcriptional, rather than transcriptional, regulation. Moreover, heterogeneous nuclear ribonucleoprotein Q (HNRNP Q) specifically binds to the 3'untranslated region of VRK2 mRNA in neuronal cells to reduce the mRNA stability. As a result, we found a dramatic decrease in CCT4 protein levels in response to a reduction in HNRNP Q levels, which was followed by an increase in polyQ aggregation in human neuroblastoma cells and mouse cortical neurons. Taken together, these results provide new insights into how neuronal HNRNP Q decreases VRK2 mRNA stability and contributes to the prevention of Huntington's disease, while also identifying new prognostic markers of HD.
Pubmed ID: 30488434 RIS Download
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This polyclonal targets GFP
View all literature mentionsThis polyclonal targets hnRNP K
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis monoclonal targets hnRNP L antibody [4D11]
View all literature mentionsThis monoclonal targets Cct4
View all literature mentionsThis monoclonal targets FLAG
View all literature mentionsThis monoclonal targets hnRNP Q clone 18E4
View all literature mentionsThis monoclonal targets VRK2 (H-5)
View all literature mentionsThis monoclonal targets VRK2 (H-255)
View all literature mentionsThis monoclonal targets GAPDH
View all literature mentionslaboratory mouse with name C57BL/6JBom from MGI.
View all literature mentionsCell line CAD is a Transformed cell line with a species of origin Mus musculus (Mouse)
View all literature mentionsCell line A-549 is a Cancer cell line with a species of origin Homo sapiens (Human)
View all literature mentionsCell line NIH 3T3 is a Spontaneously immortalized cell line with a species of origin Mus musculus
View all literature mentionslaboratory mouse with name C57BL/6JBom from MGI.
View all literature mentionsCell line NIH 3T3 is a Spontaneously immortalized cell line with a species of origin Mus musculus
View all literature mentionsThis monoclonal targets GAPDH
View all literature mentionsThis polyclonal secondary targets IgG (H+L)
View all literature mentionsThis monoclonal targets Cct4
View all literature mentionsThis monoclonal targets hnRNP Q clone 18E4
View all literature mentionsThis polyclonal targets hnRNP K
View all literature mentionsThis monoclonal targets hnRNP L antibody [4D11]
View all literature mentionsThis monoclonal targets FLAG
View all literature mentionsThis polyclonal targets GFP
View all literature mentionsThis monoclonal targets VRK2 (H-5)
View all literature mentionsThis monoclonal targets VRK2 (H-255)
View all literature mentionsCell line CAD is a Transformed cell line with a species of origin Mus musculus (Mouse)
View all literature mentionsCell line A-549 is a Cancer cell line with a species of origin Homo sapiens (Human)
View all literature mentionsThe SciCrunch Infrastructure was developed as a cooperative data platform to be used by diverse communities in making data more FAIR.
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