SPECIAL ARTICLE
- Model systems of protein-misfolding diseases reveal chaperone modifiers of proteotoxicity
Summary: Brehme and Voisine perform a comprehensive literature survey that highlights the power of model systems to unveil key chaperone modifiers of proteotoxicity with potential therapeutic implications.
RESEARCH ARTICLES
- A chemical with proven clinical safety rescues Down-syndrome-related phenotypes in through DYRK1A inhibition
Editors' choice: In vivo validation of a potent DYRK1A inhibitor, with proven clinical safety, using Down-syndrome- and Alzheimer's-disease-like models.
- Conditional deletion of AP-2β in mouse cranial neural crest results in anterior segment dysgenesis and early-onset glaucoma
Summary: Tissue-specific deletion of transcription factor AP-2β in the neural-crest-derived periocular mesenchyme generates a novel model of anterior segment dysgenesis and early onset glaucoma in mice.
- YBR/EiJ mice: a new model of glaucoma caused by genes on chromosomes 4 and 17
Summary: We identify the YBR/EiJ mouse strain as a new model of high intraocular pressure and glaucoma, and also identify genetic loci that contribute to this glaucoma.
- Loss of vhl in the zebrafish pronephros recapitulates early stages of human clear cell renal cell carcinoma
Summary: Zebrafish with an inactivating mutation in the vhl gene can be used as a model of early stage clear cell renal cell carcinoma, with applications for genetic studies and drug screens.
- Alterations in nuclear structure promote lupus autoimmunity in a mouse model
Summary: Combining a disruption in nuclear structure with a lupus-prone genetic background induces autoimmunity, suggesting that nuclear alterations trigger the disease in genetically susceptible individuals.
- MicroRNA screening identifies a link between NOVA1 expression and a low level of IKAP in familial dysautonomia
Summary: A miRNA screening conducted in olfactory stem cells from patients links the neuron-specific splicing factor NOVA1 to neurodegeneration in familial dysautonomia.
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Editor’s choice – Perturbed development of cranial neural crest cells in association with reduced sonic hedgehog signaling underlies the pathogenesis of retinoic-acid-induced cleft palate
Takashi Yamashiro and colleagues identify the critical role of the retinoic acid-Sonic hedgehog signalling pathway in cranial neural crest and palate development.
A new Resource article by Nguyen et al. describes CFIN, a novel deep learning image analysis platform to assess cardiac function in embryonic zebrafish.
Yuyu Niu and colleagues summarise the history of genome editing in non-human primates and discusses the challenges and prospects of the technology.
Have you seen our interviews with the early-career first authors of our papers? Recently, we caught up with Alexander Akerberg and Jocelyn Wessels.
Travelling Fellowship – New imaging approach unveils a bigger picture
Find out how Pamela Imperadore’s Travelling Fellowship grant from The Company of Biologists took her to Germany, where she used new imaging techniques to investigate the cellular machinery underlying octopus arm regeneration. Don’t miss the next application deadline for 2020 travel, coming up on 29 November. Where will you go?
preLights – HSP110 dependent HSP70 disaggregation machinery mediates prion-like propagation of amyloidogenic proteins in metazoa
Highlighted by Tessa Sinnige, a recent preprint from Carmen Nussbaum-Krammer and co-workers shows that Hsp110 activity protects against amorphous protein aggregation but promotes amyloid aggregation and toxicity in C. elegans models.