Dysregulated ribosome quality control in human diseases
Precise regulation of mRNA translation is of fundamental importance for maintaining
homeostasis. Conversely, dysregulated general or transcript‐specific translation, as well as …
homeostasis. Conversely, dysregulated general or transcript‐specific translation, as well as …
The eRF1 degrader SRI-41315 acts as a molecular glue at the ribosomal decoding center
Translation termination is an essential cellular process, which is also of therapeutic interest
for diseases that manifest from premature stop codons. In eukaryotes, translation termination …
for diseases that manifest from premature stop codons. In eukaryotes, translation termination …
Is there a localized role for translational quality control?
S Meydan, NR Guydosh - RNA, 2023 - rnajournal.cshlp.org
It is known that mRNAs and the machinery that translates them are not uniformly distributed
throughout the cytoplasm. As a result, the expression of some genes is localized to particular …
throughout the cytoplasm. As a result, the expression of some genes is localized to particular …
Translation velocity determines the efficacy of engineered suppressor tRNAs on pathogenic nonsense mutations
N Bharti, L Santos, M Davyt, S Behrmann… - Nature …, 2024 - nature.com
Nonsense mutations–the underlying cause of approximately 11% of all genetic diseases–
prematurely terminate protein synthesis by mutating a sense codon to a premature stop or …
prematurely terminate protein synthesis by mutating a sense codon to a premature stop or …
[HTML][HTML] RNF14-dependent atypical ubiquitylation promotes translation-coupled resolution of RNA-protein crosslinks
S Zhao, J Cordes, KM Caban, MJ Götz… - Molecular cell, 2023 - cell.com
Reactive aldehydes are abundant endogenous metabolites that challenge homeostasis by
crosslinking cellular macromolecules. Aldehyde-induced DNA damage requires repair to …
crosslinking cellular macromolecules. Aldehyde-induced DNA damage requires repair to …
K6-linked ubiquitylation marks formaldehyde-induced RNA-protein crosslinks for resolution
AS Rahmanto, CJ Blum, C Scalera, JB Heidelberger… - Molecular Cell, 2023 - cell.com
Reactive aldehydes are produced by normal cellular metabolism or after alcohol
consumption, and they accumulate in human tissues if aldehyde clearance mechanisms are …
consumption, and they accumulate in human tissues if aldehyde clearance mechanisms are …
[PDF][PDF] mRNA-specific readthrough of nonsense codons by antisense oligonucleotides (R-ASOs)
Nonsense mutations account for> 10% of human genetic disorders, including cystic fibrosis,
Alagille syndrome, and Duchenne muscular dystrophy. A nonsense mutation results in the …
Alagille syndrome, and Duchenne muscular dystrophy. A nonsense mutation results in the …
[HTML][HTML] Ubiquitin-dependent translation control mechanisms: Degradation and beyond
Translation control mechanisms connect the largely static genome to the highly dynamic
proteome. At each step in the translation cycle, multiple layers of regulation enable efficient …
proteome. At each step in the translation cycle, multiple layers of regulation enable efficient …
[HTML][HTML] Exploring the multifaceted role of GCN1: Implications in cellular responses and disease pathogenesis
X Zheng, J Shen, H Jiang, M Tian, Q Wang… - Biomedicine & …, 2024 - Elsevier
GCN1 is a highly conserved protein present widely across eukaryotes. As an upstream
activator of protein kinase GCN2, GCN1 plays a pivotal role in integrated stress responses …
activator of protein kinase GCN2, GCN1 plays a pivotal role in integrated stress responses …
Exploring the therapeutic potential of modulating nonsense-mediated mRNA decay
M McMahon, LE Maquat - RNA, 2024 - rnajournal.cshlp.org
Discovered more than four decades ago, nonsense-mediated mRNA decay (NMD) plays a
fundamental role in the regulation of gene expression and is a major contributor to …
fundamental role in the regulation of gene expression and is a major contributor to …