Molecular impact of mutations in RNA splicing factors in cancer

Q Zhang, Y Ai, O Abdel-Wahab - Molecular Cell, 2024 - cell.com
Somatic mutations in genes encoding components of the RNA splicing machinery occur
frequently in multiple forms of cancer. The most frequently mutated RNA splicing factors in …

Pre-mRNA splicing-associated diseases and therapies

SL Love, JD Emerson, K Koide, AA Hoskins - RNA biology, 2023 - Taylor & Francis
Precursor mRNA (pre-mRNA) splicing is an essential step in human gene expression and is
carried out by a large macromolecular machine called the spliceosome. Given the …

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 …

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 …

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 …

Characterization of the SF3B1–SUGP1 interface reveals how numerous cancer mutations cause mRNA missplicing

J Zhang, J **e, J Huang, X Liu, R Xu… - Genes & …, 2023 - genesdev.cshlp.org
The spliceosomal gene SF3B1 is frequently mutated in cancer. While it is known that SF3B1
hotspot mutations lead to loss of splicing factor SUGP1 from spliceosomes, the cancer …

Targeted high-throughput mutagenesis of the human spliceosome reveals its in vivo operating principles

I Beusch, B Rao, MK Studer, T Luhovska, V Šukytė… - Molecular cell, 2023 - cell.com
The spliceosome is a staggeringly complex machine, comprising, in humans, 5 snRNAs
and> 150 proteins. We scaled haploid CRISPR-Cas9 base editing to target the entire human …

[HTML][HTML] Dynamic interactions drive early spliceosome assembly

S Martínez-Lumbreras, C Morguet, M Sattler - Current Opinion in Structural …, 2024 - Elsevier
Splicing is a critical processing step during pre-mRNA maturation in eukaryotes. The correct
selection of splice sites during the early steps of spliceosome assembly is highly important …

[HTML][HTML] Splicing quality control mediated by DHX15 and its G-patch activator SUGP1

Q Feng, K Krick, J Chu, CB Burge - Cell reports, 2023 - cell.com
Pre-mRNA splicing is surveilled at different stages by quality control (QC) mechanisms. The
leukemia-associated DExH-box family helicase hDHX15/scPrp43 is known to disassemble …

[HTML][HTML] Structural basis of catalytic activation in human splicing

J Schmitzová, C Cretu, C Dienemann, H Urlaub… - Nature, 2023 - nature.com
Pre-mRNA splicing follows a pathway driven by ATP-dependent RNA helicases. A crucial
event of the splicing pathway is the catalytic activation, which takes place at the transition …