A MODEL FOR LIFE
FIRST PERSON
REVIEWS
- The roles of dystroglycan in the nervous system: insights from animal models of muscular dystrophy
Summary: Dystroglycan is a ubiquitous matrix receptor linked to brain and muscle disease. Unraveling the functions of this protein will inform basic and translational research on neural development and muscular dystrophies.
- Hypoxia and connectivity in the developing vertebrate nervous system
Summary: The nervous system's exposure to hypoxia has developmental and clinical relevance. In this Review, the authors discuss the effects of hypoxia on the development of the CNS, and its long-term behavioral and neurodevelopmental consequences.
RESEARCH ARTICLES
- A viable hypomorphic Arnt2 mutation causes hyperphagic obesity, diabetes and hepatic steatosis
Summary: A forward genetic approach identified genes involved in maintaining normal body weight. A mutation in Arnt2 caused obesity by regulating food intake.
- Dietary advanced glycation end-product consumption leads to mechanical stiffening of murine intervertebral discs
Summary: Dietary AGEs lead to sex-specific intervertebral disc structural and functional changes and may be targeted for promoting spinal health, especially in diabetes, in which AGEs form rapidly.
- Mouse Idh3a mutations cause retinal degeneration and reduced mitochondrial function
Summary: Here, we show that partial loss-of-function mutations in the Idh3a gene lead to retinal degeneration due to compromised mitochondrial function. Complete loss of Idh3a, however, is embryonically lethal.
- Leukemia development initiated by deletion of RBP-J: mouse strain, deletion efficiency and cell of origin
Summary: Using a mouse model of precursor B-cell leukemia, we have investigated influencing factors on leukemia genesis, including genetic background, cell of origin and deletion efficiency.
- Semiology, clustering, periodicity and natural history of seizures in an experimental occipital cortical epilepsy model
Summary: Tetanus-toxin-induced occipital cortical epilepsy is a rodent model of acquired focal neocortical epilepsy well-suited for preclinical evaluation of novel anti-epileptic strategies.
- Collagen COL22A1 maintains vascular stability and mutations in COL22A1 are potentially associated with intracranial aneurysms
Summary: Collagen COL22A1 is expressed in perivascular fibroblast-like cells and is required to maintain vascular stability in a zebrafish model. Mutations in COL22A1 are likely to be associated with intracranial aneurysms.
- Muscle strength deficiency and mitochondrial dysfunction in a muscular dystrophy model of Caenorhabditis elegans and its functional response to drugs
Editor's choice: Dystrophin-deficient Caenorhabditis elegans have measurably weak muscle strength and mitochondrial dysfunction, and they respond to drug treatments standard in treating human Duchenne muscular dystrophy.
- Macrophages enhance Vegfa-driven angiogenesis in an embryonic zebrafish tumour xenograft model
Summary: Zebrafish embryonic macrophages associate with the distal tips of tumour xenograft blood vessels and are required for Vegfa-driven angiogenesis.
- Effect of dietary additives on intestinal permeability in both Drosophila and a human cell co-culture
Summary: Sugar and other food additives can inhibit gut barrier function in both human cell co-cultures and Drosophila melanogaster.
RESOURCE ARTICLES
- Juvenile murine models of prediabetes and type 2 diabetes develop neuropathy
Summary: The mouse models described in this paper provide critical tools to better understand the underlying pathogenesis of prediabetic and diabetic neuropathy from youth to adulthood.
- Identifying mouse developmental essential genes using machine learning
Summary: Here, we used computer-based machine learning methodology to predict which genes in the mouse genome are essential for development, and present a database of mouse essential and non-essential genes.
- Establishment of a murine culture system for modeling the temporal progression of cranial and trunk neural crest cell differentiation
Summary: A novel method for isolating and expanding primary neural crest cells, and establishment of reproducible temporal benchmarks of differentiation, provides a potential screening platform for developmental toxicity or therapeutic capacity.
- CRISPR-Cas9 human gene replacement and phenomic characterization in Caenorhabditis elegans to understand the functional conservation of human genes and decipher variants of uncertain significance
Summary: Here, we provide a CRISPR-Cas9 human gene replacement and phenomic characterization strategy to directly replace Caenorhabditis elegans genes with their human orthologs for disease variant modeling and therapeutic screening.
PREPRINT HIGHLIGHTS
This issue
Other journals from The Company of Biologists
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.