SPECIAL ARTICLE
- Sophisticated lessons from simple organisms: appreciating the value of curiosity-driven research
Summary: Curiosity-driven research using simple organisms has and will continue to lead to fundamental discoveries about biology that are directly applicable to improving the human condition.
RESEARCH ARTICLES
- Functional assays for the assessment of the pathogenicity of variants of GOSR2, an ER-to-Golgi SNARE involved in progressive myoclonus epilepsies
Summary: Mutations in the Qb-SNARE GOSR2 cause progressive myoclonus epilepsies. The authors report the effect of two mutations on SNARE function to investigate their correlation with progression and severity of disease.
- Chronic psychosocial stress disturbs long-bone growth in adolescent mice
Summary: Chronic psychosocial stress is a known risk factor for many somatic disorders. The authors demonstrate that chronic psychosocial stress negatively affects endochondral ossification in the growth plate, as well as long-bone growth. This article has an associated First Person interview with the first author of the paper as part of the supplementary information.
- Evaluation of human dermal fibroblasts directly reprogrammed to adipocyte-like cells as a metabolic disease model
Summary: Human dermal fibroblasts can be reprogrammed efficiently to adipocyte-like cells that have utility for modelling some aspects of human monogenic metabolic disease.
- Early neonatal loss of inhibitory synaptic input to the spinal motor neurons confers spina bifida-like leg dysfunction in a chicken model
Summary: Consistent with the symptoms of humans with SBA, chickens with SBA-like features exhibit lower-limb paralysis within two weeks after hatching, which appeared to coincide with synaptic change-induced disruption of spinal motor networks.
- Inclusion criteria update for the rat intraluminal ischaemic model for preclinical studies
Summary: Laser Doppler monitoring in combination with diffusion-weighted imaging and magnetic resonance angiography represents a reliable inclusion protocol during ischaemic surgery for the analysis of protective drugs in the acute phase of stroke.
- Mecp2 regulates tnfa during zebrafish embryonic development and acute inflammation
Summary: As shown by evaluating the levels of pro- and anti-inflammatory cytokines in mecp2-null zebrafish, Mecp2 is required for tnfa expression during zebrafish development and inflammation.
- A Drosophila model of insulin resistance associated with the human TRIB3 Q/R polymorphism
Summary: The insulin signaling inhibitor tribbles 3 (TRIB3) variant associated with Type II diabetes has parallel effects in a fly model system on Tribbles-regulated insulin signaling, growth and Akt activation.
- Rescue of ATXN3 neuronal toxicity in Caenorhabditis elegans by chemical modification of endoplasmic reticulum stress
Summary: We introduce a novel C. elegans model for Machado–Joseph disease for use in preclinical drug discovery and identified guanabenz as a potent neuroprotective molecule.
- Lyplal1 is dispensable for normal fat deposition in mice
Summary: We demonstrate that the Lyplal1 gene is dispensable in mice, with important implications for interpretation of GWAS results linking Lyplal1 to metabolism and fat distribution. This article has an associated First Person interview with the first author of the paper as part of the supplementary information.
- Systemic HIV-1 infection produces a unique glial footprint in humanized mouse brains
Summary: In mice with a humanized brain and immune system, systemic infection led to human-specific transcriptional induction of glial interferon antiviral innate immune pathways and alteration of neuronal progenitor differentiation and myelination.
- Renal carcinoma/kidney progenitor cell chimera organoid as a novel tumorigenesis gene discovery model
Editor’s Choice: Chimeras between embryonic kidney cells and renal carcinoma cells serve as a novel model to assay the roles of co-regulated genes in kidney development and renal carcinogenesis.
- Noncanonical NF-κB signaling and the essential kinase NIK modulate crucial features associated with eosinophilic esophagitis pathogenesis
Summary: Analyses of Nik–/– mice and human gene expression data reveal a new pathway involved in the development of eosinophilic esophagitis in human patients and mouse models of the disease. This article has an associated First Person interview with the first author of the paper as part of the supplementary information.
- Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit in the KIKO mouse model of Friedreich ataxia
Summary: Early VGLUT1-specific parallel fiber synaptic deficits and dysregulated cerebellar circuit might be potential mediators of cerebellar dysfunction and ataxia in the KIKO mouse model of Friedreich ataxia.
CORRESPONDENCE
This issue
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First person — Andrew Scott Emmert and Eri Iwasawa
In an interview, first authors Andrew Scott Emmert and Eri Iwasawa discuss their new paper on impaired CSF flow and neural differentiation in a rat model of hydrocephalus, their research process and plans for the future.
Ethan Perlstein and colleagues present two back-to-back articles. In the first, they describe how the diabetic neuropathy drug palrestat can ameliorate PMM2-CDG. The second identifies the FDA-approved aripiprazole as a clinical candidate for treating NGLY1 deficiency.
Read Murgia et al.’s new resource article describing a SILAC-based proteomics approach to quantify the unfolded protein response.
We're excited to be an affiliate journal for Review Commons, the ASAPbio/EMBO platform for high-quality journal-independent peer-review in the life sciences, which went live on 09 December.