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ProteomicsRiluzole partially restores RNA polymerase III complex assembly in cells expressing the leukodystrophy-causative variant POLR3B R103H
The mechanism of assembly of RNA polymerase III (Pol III), the 17-subunit enzyme that synthesizes tRNAs, 5S rRNA, and other small-nuclear (sn) RNAs in eukaryotes, is not clearly understood. The recent discovery of the HSP90 co-chaperone PAQosome (Particle for Arrangement of Quaternary structure) rev...
ORGANISM(S): Homo sapiens (Human) 
2023-03-10 | PXD034961 | Pride
Protein complex assembly Rna polymerase iii Mass spectrometry Paqosome Riluzole Polr3-related leukodystrophy 4h leukodystrophy Drug repurposing 
TranscriptomicsThe RPB2 flap loop of human RNA polymerase II is dispensable for transcription initiation and elongation.
Deletion of the flap loop in the Rp2 subunit of human RNAPII did not alter the ability of human RNAPII to initiate transcription or elongate the initiated transcripts in vivo and in vitro, and was also dispensable for elongation factor-mediated enhancement and inhibition of transcript elongation in ...
ORGANISM(S): Homo sapiens 
2011-06-24 | E-GEOD-29734 | biostudies-arrayexpress
ProteomicsThe unphosphorylated form of the PAQosome core subunit RPAP3 binds ribosomal preassembly complexes to modulate ribosome biogenesis.
The PAQosome (Particle for Arrangement of Quaternary structure) is a twelve-subunit HSP90 co-chaperone involved in the biogenesis of several human protein complexes. Two mechanisms of client selection have previously been identified, namely the selective recruitment of specific adaptors and the diff...
ORGANISM(S): Homo sapiens (Human) 
2022-06-27 | PXD026647 | Pride
Ap-ms Ribosome preassembly factors Rpap3 Ribosome biogenesis Chaperone Ck2 phosphorylation Paqosome 
ProteomicsUpstream ORF-encoded ASDURF is a novel prefoldin-like subunit of the PAQosome
The PAQosome is an eleven-subunit chaperone involved in the biogenesis of several human protein complexes. We show that ASDURF, a recently discovered upstream open reading frame (uORF) in the 5’ UTR of ASNSD1 mRNA, encodes the twelfth subunit of the PAQosome. ASDURF, displays significant structural ...
ORGANISM(S): Homo sapiens (Human) 
2019-11-25 | PXD015848 | Pride
Ap-ms Protein complex assembly Uorf Paqosome Small orf encoded peptide Chaperone Asdurf Bioid R2tp/pfdl Prefoldin 
ProteomicsKIN interactome - Methylation of the DNA/RNA-binding protein Kin17 by METTL22 affects its association with chromatin
BioID experiment of KIN protein to determine its interactors
ORGANISM(S): Homo sapiens (Human) 
2019-10-16 | PXD010487 | Pride
Kin Cancer Splicing process Protein-protein interaction Ribosome biogenesis Bioid 
ProteomicsR2TP_BioID-MS - R2TP/Prefoldin-like component RUVBL1/RUVBL2 directly interacts with ZNHIT2 to regulate assembly of U5 small nuclear ribonucleoprotein
The R2TP/Prefoldin-like (R2TP/PFDL) complex is a cochaperone complex involved in the assembly of a number of critical protein complexes. Here we use proximity-dependent biotinylation (BioID) coupled to mass spectrometry to confirm interactions of ZNHIT2, a cofactor of R2TP/PFDN, identified by TAP-MS...
ORGANISM(S): Homo sapiens (Human) 
2018-10-24 | PXD006200 | Pride
Human Lc-msms U5 snrnp Flp-in t-rex hek 293 Znhit2 R2tp/pfdn Bioid 
ProteomicsR2TP_SILAC_FLAG-MS - R2TP/Prefoldin-like component RUVBL1/RUVBL2 directly interacts with ZNHIT2 to regulate assembly of U5 small nuclear ribonucleoprotein
The R2TP/Prefoldin-like (R2TP/PFDL) complex is a cochaperone complex involved in the assembly of a number of critical protein complexes. Here we use stable isotope labeling with amino acids in cell culture (SILAC) and FLAG tag affinity purification coupled to mass spectrometry to assess whether siRN...
ORGANISM(S): Homo sapiens (Human) 
2017-11-02 | PXD006199 | Pride
Human Lc-msms Tsc complex Affinity purification U5 snrnp Znhit2 Hek 293 Flag 
ProteomicsR2TP_TAP-MS - R2TP/Prefoldin-like component RUVBL1/RUVBL2 directly interacts with ZNHIT2 to regulate assembly of U5 small nuclear ribonucleoprotein
The R2TP/Prefoldin-like (R2TP/PFDL) complex is a cochaperone complex involved in the assembly of a number of critical protein complexes. Here we use multiple target tandem affinity purification (TAP) coupled to mass spectrometry to map out the interaction network around R2TP/PFDL.
ORGANISM(S): Homo sapiens (Human) 
2018-10-24 | PXD006198 | Pride
Human Lc-msms Tap Hek 293 
ProteomicsRecessive mutations in POLR1C cause a leukodystrophy by impairing biogenesis of RNA polymerase III
A small proportion of 4H (Hypomyelination, Hypodontia and Hypogonadotropic Hypogonadism) or RNA polymerase III (POLR3)-related leukodystrophy cases are negative for mutations in the previously identified causative genes POLR3A and POLR3B. Here, we report eight of these cases carrying recessive mutat...
ORGANISM(S): Homo sapiens (Human) 
2019-10-16 | PXD002154 | Pride
Leukodystrophy Human Rna polymerase iii Polr1c 
TranscriptomicsChromatin immunoprecipitation of human cell lines with oestrogen receptor (ER), the E2F transcription factor 4 (E2F4),
The Goal of the experiment was to validate the predicted regulatory modules identified by an algorithm.For this we took advantage of the the genome-wide location analysis technique (or ?ChIP/chip?). We selected modules predicted to be bound by the estrogen receptor (ER), the E2F transcription factor...
ORGANISM(S): Homo sapiens 
2006-05-01 | E-MEXP-471 | biostudies-arrayexpress
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