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
DMM and COVID-19
We are aware that the COVID-19 pandemic is having an unprecedented impact on researchers worldwide. The Editors of all The Company of Biologists’ journals have been considering ways in which we can alleviate concerns that members of our community may have around publishing activities during this time. Read about the actions we are taking at this time.
Please don’t hesitate to contact the Editorial Office if you have any questions or concerns.
A Model for Life – Elizabeth McNally and Louis Kunkel
Human geneticist and cardiologist Elizabeth McNally discusses her first experiences in science and the effort we all need to make to facilitate diversity in science, while Louis Kunkel tells us about talks about his discovery of dystrophin.
On the cover of our special issue, Demonbreun et al. present muscle fiber typing from a newly created mouse model of limb-girdle muscular dystrophy generated by gene editing.
Non-canonical autophagy in innate immune defence in vivo
preLighter Kirsty Hooper, The Babraham Institute, Cambridge, UK, discusses a preprint showing that loss of non-canonical autophagy in mice increases sensitivity to influenza A virus infection.
The Biology Open transfer option
DMM authors can transfer their submission to our sister journal Biology Open with just one click. An online Open Access journal, BiO also welcomes direct submissions and focuses on the swift publication of high-quality, sound research. Recent disease modelling highlights in BiO include work by Liu et al. investigating transgenic replenishment strategies targeting myocardial and non-myocardial tissues in the zebrafish. Find out more about how to transfer.
Editor’s choice
Highlighted by the Guest editors of our special issue, Grunwald and colleagues use zebrafish to investigate interactions among ryanodine receptor isotypes in muscle development and function, while Claire Wood et al. tests the mdx:Cmah−/− mouse to investigate growth and skeletal development in Duchenne muscular dystrophy.