Sex-dependent effect of APOE on Alzheimer's disease and other age-related neurodegenerative disorders
Summary: The APOE ε4 allele and female sex are among the strongest risk factors for late-onset Alzheimer's disease. We explore how the interplay between these factors affects risk for this and other age-related neurodegenerative diseases.
Summary: In this Review, we discuss how zebrafish (Danio rerio) and other fish models can complement the more traditional mammalian models in the development of novel vaccines against tuberculosis.
Loss of the Drosophila branched-chain α-ketoacid dehydrogenase complex results in neuronal dysfunction
Summary: Loss of BCKDH activity in Drosophila recapitulates the neurological symptoms of patients with maple syrup urine disease. Metformin administration was found to alleviate developmental defects and aberrant behavior in the BCKDH mutant.
A regulated NMD mouse model supports NMD inhibition as a viable therapeutic option to treat genetic diseases
Summary: Nonsense-mediated mRNA decay can be inhibited after completion of mammalian prenatal development without adverse effects in non-neurological, somatic tissues, indicating that such inhibition might be a viable therapeutic strategy.
Summary: Using maltodextrin-dominant human infant formula feeding in combination with hypoxia, we developed a highly reproducible model of small intestinal injury in the neonatal mouse.
Generation and characterization of an Il2rg knockout Syrian hamster model for XSCID and HAdV-C6 infection in immunocompromised patients
Summary: Syrian hamsters are an important rodent species for studying multiple human diseases. We describe the generation and characterization of a hamster strain that is defective in the XCSID-associated IL2RG gene.
suz12 inactivation in p53- and nf1-deficient zebrafish accelerates the onset of malignant peripheral nerve sheath tumors and expands the spectrum of tumor types
Summary: In p53- and nf1-deficient zebrafish, onset of MPNSTs, as well as diverse other tumors, is accelerated by loss of the suz12 tumor suppressor, accompanied by global reduction in H3K27me3 marks and increased Ras-Mapk signaling.
Nrf2/HO-1 mediates the neuroprotective effects of pramipexole by attenuating oxidative damage and mitochondrial perturbation after traumatic brain injury in rats
Editor's choice: We administered pramipexole (PPX) to rats after a traumatic brain injury (TBI) and evaluated various parameters, which indicate that the neuroprotective effects of PPX are mediated by activation of the Nrf2/HO-1 signaling pathway following TBI.
Summary: Our study has uncovered Maz as an important regulator of eye development in humans and mice, striving to elucidate the role of this gene in eye abnormalities associated with the human ch16p11.2 microdeletions and microduplications.
Systemic and heart autonomous effects of sphingosine Δ4 desaturase deficiency in lipotoxic cardiac pathophysiology
Summary: Systemic versus heart autonomous functions of sphingosine Δ4 desaturase differentially regulate cardiac structure and function in a tissue-specific manner in Drosophila, where organ interplay mimics that observed in mammalian systems.