Cellular functions of eukaryotic RNA helicases and their links to human diseases

KE Bohnsack, S Yi, S Venus, E Jankowsky… - … Reviews Molecular Cell …, 2023 - nature.com
RNA helicases are highly conserved proteins that use nucleoside triphosphates to bind or
remodel RNA, RNA–protein complexes or both. RNA helicases are classified into the DEAD …

Genetics of 46, XY gonadal dysgenesis

M Elzaiat, K McElreavey, A Bashamboo - Best Practice & Research Clinical …, 2022 - Elsevier
In 46, XY men, testis is determined by a genetic network (s) that both promotes testis
formation and represses ovarian development. Disruption of this process results in a lack of …

DHX15 is involved in SUGP1-mediated RNA missplicing by mutant SF3B1 in cancer

J Zhang, J Huang, K Xu, P **ng… - Proceedings of the …, 2022 - National Acad Sciences
SF3B1 is the most frequently mutated spliceosomal gene in cancer. Several hotspot
mutations are known to disrupt the interaction of SF3B1 with another splicing factor, SUGP1 …

RNA folding and functions of RNA helicases in ribosome biogenesis

V Mitterer, B Pertschy - RNA biology, 2022 - Taylor & Francis
Eukaryotic ribosome biogenesis involves the synthesis of ribosomal RNA (rRNA) and its
stepwise folding into the unique structure present in mature ribosomes. rRNA folding starts …

Mechanism for the initiation of spliceosome disassembly

MK Vorländer, P Rothe, J Kleifeld, ED Cormack… - Nature, 2024 - nature.com
Abstract Precursor-mRNA (pre-mRNA) splicing requires the assembly, remodelling and
disassembly of the multi-megadalton ribonucleoprotein complex called the spliceosome …

Prp43/DHX15 exemplify RNA helicase multifunctionality in the gene expression network

KE Bohnsack, N Kanwal, MT Bohnsack - Nucleic acids research, 2022 - academic.oup.com
Dynamic regulation of RNA folding and structure is critical for the biogenesis and function of
RNAs and ribonucleoprotein (RNP) complexes. Through their nucleotide triphosphate …

A super minigene with a short promoter and truncated introns recapitulates essential features of transcription and splicing regulation of the SMN1 and SMN2 genes

EW Ottesen, J Seo, D Luo, NN Singh… - Nucleic Acids …, 2024 - academic.oup.com
Here we report a Survival Motor Neuron 2 (SMN2) super minigene, SMN2Sup,
encompassing its own promoter, all exons, their flanking intronic sequences and the entire …

The splicing regulators RBM5 and RBM10 are subunits of the U2 snRNP engaged with intron branch sites on chromatin

A Damianov, CH Lin, J Huang, L Zhou… - Molecular Cell, 2024 - cell.com
Understanding the mechanisms of pre-mRNA splicing is limited by the technical challenges
to examining spliceosomes in vivo. Here, we report the isolation of RNP complexes derived …

GPATCH8 modulates mutant SF3B1 mis-splicing and pathogenicity in hematologic malignancies

S Benbarche, JMB Pineda, LB Galvis, J Biswas, B Liu… - Molecular Cell, 2024 - cell.com
Mutations in the RNA splicing factor gene SF3B1 are common across hematologic and solid
cancers and result in widespread alterations in splicing, yet there is currently no therapeutic …

Regulation of the DEAH/RHA helicase Prp43 by the G-patch factor Pfa1

M Enders, R Ficner, S Adio - Proceedings of the National …, 2022 - National Acad Sciences
The DEAH/RHA helicase Prp43 remodels protein–RNA complexes during pre-messenger
RNA (mRNA) splicing and ribosome biogenesis. The helicase activity and ATP turnover are …