Summary: Gastroenteropancreatic neuroendocrine neoplasms have been an understudied entity with limited treatment options. We discuss the disease models, including emerging organoids, that may provide biological and therapeutic insight into these diseases.
Summary: Microvillus inclusion disease serves as a useful model to enhance our understanding of the intestinal trafficking and polarity machinery in health and disease.
Developmental abnormalities in supporting cell phalangeal processes and cytoskeleton in the Gjb2 knockdown mouse model
Summary: A reduction in connexin 26 before opening of the tunnel of Corti impedes microtubule formation in supporting cells, and this may lead to cochlear developmental abnormalities and deafness in the Gjb2 knockdown mouse model.
Summary: Loss of RNA splicing factors causes R-loop accumulation and DNA damage in embryonic neurons, sensitizing them to radiation-induced cell death. These findings suggest that diseased cells with mutations in splicing factors are vulnerable to radiotherapy.
Inorganic arsenic causes fatty liver and interacts with ethanol to cause alcoholic liver disease in zebrafish
Summary: Using zebrafish, the authors show that exposure to a common environmental contaminant, inorganic arsenic, increases the risk of alcoholic liver disease.
Summary: In this manuscript, we discovered motor neuron degeneration which correlates with respiratory failure in a knock-in mouse model of spinocerebellar ataxia type 1 (SCA1).
Divergent effects of adrenaline in human induced pluripotent stem cell-derived cardiomyocytes obtained from hypertrophic cardiomyopathy
Summary: Different concentrations of adrenaline have divergent effects during and immediately after administration in human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) obtained from hypertrophic cardiomyopathy (HCM) patients. Bisoprolol could not reduce the arrhythmias in HCM-specific hiPSC-CMs.
The IgCAM CLMP regulates expression of Connexin43 and Connexin45 in intestinal and ureteral smooth muscle contraction in mice
Summary: The function of the immunoglobulin cell adhesion molecule CLMP was investigated in a mouse model. CLMP is essential for intestinal and ureteral peristalsis, and for expression of Connexin43 and 45 in smooth muscle cells.
Summary: Ubiquitous alteration of choroid circulation causes localized retinal alterations in mice that are similar to human non-exudative age-related macular degeneration, thus providing a new potential experimental model of the disease.
Environmental factors regulate Paneth cell phenotype and host susceptibility to intestinal inflammation in Irgm1-deficient mice
Summary: In this study, we rederived conventionally raised Irgm1-deficient mice into specific pathogen-free and germ-free conditions. We show that these environments determine how Irgm1 regulates Paneth cell function and gut inflammation susceptibility.
Inhibition of galectin-3 ameliorates the consequences of cardiac lipotoxicity in a rat model of diet-induced obesity
Summary: Inhibition of Gal-3 activity reduced the excessive cardiac accumulation of lipids in rats fed a high fat diet. This was accompanied by the amelioration of mitochondria damage observed in obese rats.
Editor's choice: A new ex vivo experimental approach to explore how glioblastoma stem cells interact with distinct brain microenvironments, with potential use a as preclinical model using new anticancer agents.
High-throughput screen for compounds that modulate neurite growth of human induced pluripotent stem cell-derived neurons
Summary: High-throughput, small molecule screening of hiPSC-derived neurons using a high-content, image-based approach focused on neurite growth identified hit compounds, including approved drugs, which target molecules or pathways known to regulate neurite growth.