Mechanisms, regulation and functions of the unfolded protein response

C Hetz, K Zhang, RJ Kaufman - Nature reviews Molecular cell biology, 2020 - nature.com
Cellular stress induced by the abnormal accumulation of unfolded or misfolded proteins at
the endoplasmic reticulum (ER) is emerging as a possible driver of human diseases …

RNA modifications regulating cell fate in cancer

S Delaunay, M Frye - Nature cell biology, 2019 - nature.com
The deposition of chemical modifications into RNA is a crucial regulator of temporal and
spatial gene expression programs during development. Accordingly, altered RNA …

Cell stress in cortical organoids impairs molecular subtype specification

A Bhaduri, MG Andrews, W Mancia Leon, D Jung… - Nature, 2020 - nature.com
Cortical organoids are self-organizing three-dimensional cultures that model features of the
develo** human cerebral cortex,. However, the fidelity of organoid models remains …

Codon-specific translation reprogramming promotes resistance to targeted therapy

F Rapino, S Delaunay, F Rambow, Z Zhou, L Tharun… - Nature, 2018 - nature.com
Reprogramming of mRNA translation has a key role in cancer development and drug
resistance. However, the molecular mechanisms that are involved in this process remain …

Germline elongator mutations in sonic hedgehog medulloblastoma

SM Waszak, GW Robinson, BL Gudenas, KS Smith… - Nature, 2020 - nature.com
Cancer genomics has revealed many genes and core molecular processes that contribute to
human malignancies, but the genetic and molecular bases of many rare cancers remains …

Applications of brain organoids in neurodevelopment and neurological diseases

N Sun, X Meng, Y Liu, D Song, C Jiang… - Journal of Biomedical …, 2021 - Springer
A brain organoid is a self-organizing three-dimensional tissue derived from human
embryonic stem cells or pluripotent stem cells and is able to simulate the architecture and …

Adapting the proteostasis capacity to sustain brain healthspan

C Hetz - Cell, 2021 - cell.com
Sustaining neuronal proteostasis during the course of our life is a central aspect required for
brain function. The dynamic nature of synaptic composition and abundance is a requisite to …

Mutations in KEOPS-complex genes cause nephrotic syndrome with primary microcephaly

DA Braun, J Rao, G Mollet, D Schapiro, MC Daugeron… - Nature …, 2017 - nature.com
Galloway–Mowat syndrome (GAMOS) is an autosomal-recessive disease characterized by
the combination of early-onset nephrotic syndrome (SRNS) and microcephaly with brain …

Queuosine‐modified tRNAs confer nutritional control of protein translation

F Tuorto, C Legrand, C Cirzi, G Federico… - The EMBO …, 2018 - embopress.org
Global protein translation as well as translation at the codon level can be regulated by tRNA
modifications. In eukaryotes, levels of tRNA queuosinylation reflect the bioavailability of the …

Human 3D cellular model of hypoxic brain injury of prematurity

AM Pașca, JY Park, HW Shin, Q Qi, O Revah… - Nature medicine, 2019 - nature.com
Owing to recent medical and technological advances in neonatal care, infants born
extremely premature have increased survival rates,. After birth, these infants are at high risk …