Modeling neurodegenerative disorders in zebrafish

AA Bashirzade, KN Zabegalov, AD Volgin… - Neuroscience & …, 2022 - Elsevier
Neurodegeneration is a major cause of Alzheimer's, Parkinson's, Huntington's, multiple and
amyotrophic lateral sclerosis, pontocerebellar hypoplasia, dementia and other related brain …

Recent insights into the structure, function, and regulation of the eukaryotic transfer RNA splicing endonuclease complex

CK Hayne, TA Lewis, RE Stanley - Wiley Interdisciplinary …, 2022 - Wiley Online Library
The splicing of transfer RNA (tRNA) introns is a critical step of tRNA maturation, for intron‐
containing tRNAs. In eukaryotes, tRNA splicing is a multi‐step process that relies on several …

[HTML][HTML] Trehalose treatment in zebrafish model of Lafora disease

S Della Vecchia, A Ogi, R Licitra, F Abramo… - International Journal of …, 2022 - mdpi.com
Mutations in the EPM2A gene encoding laforin cause Lafora disease (LD), a progressive
myoclonic epilepsy characterized by drug-resistant seizures and progressive neurological …

A high-throughput assay for directly monitoring nucleolar rRNA biogenesis

CJ Bryant, MA McCool, L Abriola… - Open …, 2022 - royalsocietypublishing.org
Studies of the regulation of nucleolar function are critical for ascertaining clearer insights into
the basic biological underpinnings of ribosome biogenesis (RB), and for future development …

[HTML][HTML] RNA exosome component EXOSC4 amplified in multiple cancer types is required for the cancer cell survival

K Taniue, T Tanu, Y Shimoura, S Mitsutomi… - International journal of …, 2022 - mdpi.com
The RNA exosome is a multi-subunit ribonuclease complex that is evolutionally conserved
and the major cellular machinery for the surveillance, processing, degradation, and turnover …

EXOSC8 promotes colorectal cancer tumorigenesis via regulating ribosome biogenesis-related processes

K Cui, L Gong, H Zhang, Y Chen, B Liu, Z Gong, J Li… - Oncogene, 2022 - nature.com
Extensive protein synthesis is necessary for uncontrolled cancer cell proliferation, requiring
hyperactive ribosome biogenesis. Our previous Pan-cancer study has identified EXOSC8 as …

Bi‐allelic missense variant, p.Ser35Leu in EXOSC1 is associated with pontocerebellar hypoplasia

PH Somashekar, P Kaur, J Stephen… - Clinical …, 2021 - Wiley Online Library
RNA exosome is a highly conserved ribonuclease complex essential for RNA processing
and degradation. Bi‐allelic variants in exosome subunits EXOSC3, EXOSC8 and EXOSC9 …

mRNA isoform balance in neuronal development and disease

GR LaForce, P Philippidou… - Wiley Interdisciplinary …, 2023 - Wiley Online Library
Balanced mRNA isoform diversity and abundance are spatially and temporally regulated
throughout cellular differentiation. The proportion of expressed isoforms contributes to cell …

Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study

V Brocklebank, PR Walsh, K Smith-Jackson… - Blood, 2023 - ashpublications.org
Historically, the majority of patients with complement-mediated atypical hemolytic uremic
syndrome (CaHUS) progress to end-stage kidney disease (ESKD). Single-arm trials of …

Rcl1 depletion impairs 18S pre-rRNA processing at the A1-site and up-regulates a cohort of ribosome biogenesis genes in zebrafish

Q Zhu, B Tao, H Chen, H Shi, L Huang… - Nucleic Acids …, 2021 - academic.oup.com
Yeast Rcl1 is a potential endonuclease that mediates pre-RNA cleavage at the A2-site to
separate 18S rRNA from 5.8 S and 25S rRNAs. However, the biological function of Rcl1 in …