Reciprocal and non-reciprocal effects of clinically relevant SETBP1 protein dosage changes.

Many genes in the human genome encode proteins that are dosage sensitive, meaning they require protein levels within a narrow range to properly execute function. To investigate if clinically relevant variation in protein levels impacts the same downstream pathways in human disease, we generated cell models of two SETBP1 syndromes: Schinzel-Giedion Syndrome (SGS) and SETBP1 haploinsufficiency disease (SHD), where SGS is caused by too much protein, and SHD is caused by not enough SETBP1. Using patient and sex-matched healthy first-degree relatives from both SGS and SHD SETBP1 cases, we assessed how SETBP1 protein dosage affects downstream pathways in human forebrain progenitor cells.

Advances in the Assessment and Treatment of Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases primarily cause inflammation of small blood vessels. Renal involvement occurs frequently and often leads to end-stage renal disease (ESRD), which significantly impacts patient health and survival. Early diagnosis and appropriate treatment are essential to improving patient outcomes.

Chronic traumatic brain injury induces neurodegeneration, neuroinflammation, and cognitive deficits in a T cell-dependent manner.

Traumatic brain injury (TBI) can lead to chronic neuroinflammation, and neurodegeneration associated with long-term cognitive deficits. Following TBI, the acute neuroinflammatory response involves microglial activation and the release of proinflammatory cytokines and chemokines which induce the recruitment of peripheral immune cells such as monocytes and ultimately T cells. Persistent innate and adaptive immune cell responses can lead to chronic neurodegeneration and functional deficits.

Structure-based design of covalent nanobody binders for a thermostable green fluorescence protein.

The use of green fluorescence protein (GFP) has advanced numerous areas of life sciences. An ultra-thermostable GFP (TGP), engineered from a coral GFP, offers potential advantages over traditional jellyfish-derived GFP because of its high stability. However, owing to its later discovery, TGP lacks the extensive toolsets available for GFP, such as heavy chain-only antibody binders known as nanobodies.

Preparation of Flame-Retardant Glass Fiber-Reinforced Epoxy by Vacuum-Assisted Resin Infusion Process with Deep Eutectic Point Curing Agent.

Enhancing the flame resistance of fiber-reinforced epoxy resins will broaden its potential applications. However, the inclusion of a flame retardant in epoxy resins with a curing agent shortens the process, posing a challenge for the production of flame-retardant composites. In this work, the process performance of flame-retardant epoxy resins was improved by using deep eutectic solvents (DES) as the curing agent.