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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First person – Zhaoyang, Omkara, Christine and Yong
Catch up with our first authors in the latest interviews. Zhaoyang Liu, Omkara Lakshmi Veeranki, Christine Lehner and Yong Chen discuss the implications of their research, life as an early-career researcher and their next steps.
A new resource article by Dipen Maru's group describes a patient-derived orthotopic xenograft model of gastroesphageal cancer that maintains the morphological and molecular characteristics of the source tumour.
There are still funded places available for early-career researchers at July’s Workshop on inflammaging and regeneration! The deadline for applications is 14 February. We’re also interested to hear your ideas for Workshops – find out more about how we can provide support.
The first preLight of 2020 and the first time we’ve highlighted a medRxiv preprint! Zhang-He Goh discusses a new preprint by Witte et al. assessing the correlation between ovarian cancer patient-derived organoids and patients’ clinical response to chemotherapeutics.
Cover article – Regulation of terminal hypertrophic chondrocyte differentiation in Prmt5 mutant mice modeling infantile idiopathic scoliosis
On the cover, research by Liu et al. demonstrates that the loss of Prmt5 osteochondral progenitors impairs terminal hypertrophic chondrocyte differentiation, leading to defects in endochondral bone formation and models infantile idiopathic scoliosis in mouse.