ISG15 protects human Tregs from interferon alpha‐induced contraction in a cell‐intrinsic fashion
Objectives Type I interferons (IFNs) inhibit regulatory T‐cell (Treg) expansion and activation, making them beneficial in antiviral responses, but detrimental in autoimmune diseases.
Ilenia Pacella +14 more
doaj +1 more source
Impaired intrinsic immunity to HSV-1 in human iPSC-derived TLR3-deficient CNS cells [PDF]
In the course of primary infection with herpes simplex virus 1 (HSV-1), children with inborn errors of TLR3 immunity are prone to HSV-1 encephalitis (HSE) 1–3.
Abel, Laurent +22 more
core +1 more source
Management and Modification of Flavonoids Against Nonalcoholic Fatty Liver Disease
Flavonoids protect against NAFLD by enhancing mitochondrial function, reducing ER stress, and modulating gut microbiota. Structural modifications improve flavonoid efficacy, offering a promising dietary strategy for NAFLD management. ABSTRACT Flavonoids are small bioactive molecules commonly found in plant‐derived foods, exhibiting antioxidant, anti ...
Weidong Bai +6 more
wiley +1 more source
The study of inborn errors of immunity is based on a comprehensive clinical description of the patient’s phenotype and the elucidation of the underlying molecular mechanisms and their genetic etiology.
Barbara Bosch +2 more
doaj +1 more source
Rise and fall of Achille de Giovanni\u2019s clinical anthropometry [PDF]
Achille de Giovanni (1838-1916), Italian clinician and pathologist, developed a constitutional method for clinical investigations based on the morphology of the human body.
Zampieri, Fabio
core
Targeted NGS Yields Plentiful Ultra-Rare Variants in Inborn Errors of Immunity Patients [PDF]
Alice Grossi +13 more
openalex +1 more source
Immune- and nonimmune-compartment-specific interferon responses are critical determinants of herpes simplex virus-induced generalized infections and acute liver failure [PDF]
The interferon (IFN) response to viral pathogens is critical for host survival. In humans and mouse models, defects in IFN responses can result in lethal herpes simplex virus 1 (HSV-1) infections, usually from encephalitis.
Leib, David A +3 more
core +3 more sources
Porto‐sinusoidal vascular disorder in a pediatric patient with prolidase deficiency: A case report
Abstract Prolidase deficiency (PD) is a rare autosomal recessive disorder affecting collagen turnover, leading to diverse clinical manifestations including dermatologic lesions, hepatosplenomegaly, and vascular anomalies. Liver involvement in PD is poorly understood, with few reported cases.
Melissa Castro +5 more
wiley +1 more source
EBMT/ESID inborn errors working party guidelines for hematopoietic stem cell transplantation for inborn errors of immunity [PDF]
Arjan C. Lankester +11 more
openalex +1 more source
Acquired pelvic splenosis in a pediatric patient with inflammatory bowel disease: A rare case report
Abstract Splenosis is an acquired condition in which splenic tissue becomes implanted and grows in abnormal locations within the body, usually after the spleen has been injured or surgically removed. It is often discovered incidentally due to its asymptomatic nature.
Brad Pasternak +2 more
wiley +1 more source

