Ivan J. Fuss
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View article: LRRK2-mediated NLRC4 phosphorylation differentially regulates IL-1β/IL-18 secretion
LRRK2-mediated NLRC4 phosphorylation differentially regulates IL-1β/IL-18 secretion Open
In the present study, we explored the relation of LRRK2-kinase phosphorylation of the NLRC4 inflammasome to NLRC4 inflammasome function in normal humans and mice, as well as in patients with Crohn’s disease (CD). We found that LRRK2-kinase…
View article: Norovirus Infection at the NIH Clinical Center from 2010 to 2023: A Comparative Analysis of Acute vs. Chronic Norovirus Infection with a Focus on Inborn Errors of Immunity
Norovirus Infection at the NIH Clinical Center from 2010 to 2023: A Comparative Analysis of Acute vs. Chronic Norovirus Infection with a Focus on Inborn Errors of Immunity Open
Rationale Chronic norovirus infection (CNI) is a significant cause of morbidity and mortality in immunocompromised patients. We sought to determine clinical and virologic features associated with CNI with an emphasis on IEI. Methods Norovi…
View article: BTK drives neutrophil activation for sterilizing antifungal immunity
BTK drives neutrophil activation for sterilizing antifungal immunity Open
We describe a previously unappreciated role for Bruton's tyrosine kinase (BTK) in fungal immune surveillance against aspergillosis, an unforeseen complication of BTK inhibitors (BTKi) used for treating B cell lymphoid malignancies. We stud…
View article: Evaluating the efficacy and safety of pozelimab in patients with CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and protein-losing enteropathy disease: an open-label phase 2 and 3 study
Evaluating the efficacy and safety of pozelimab in patients with CD55 deficiency with hyperactivation of complement, angiopathic thrombosis, and protein-losing enteropathy disease: an open-label phase 2 and 3 study Open
View article: Inhibition of LRRK2 Kinase Activity with a Novel Inhibitor (CS 82) Inhibits NLRC4 Inflammasome Activation and Reduces Intestinal Inflammation Severity
Inhibition of LRRK2 Kinase Activity with a Novel Inhibitor (CS 82) Inhibits NLRC4 Inflammasome Activation and Reduces Intestinal Inflammation Severity Open
In the last twenty years Leucine Rich Repeat Kinase 2 (LRRK2) has received immense attention due to its association with several diseases, for example, neurodegenerative disease PD (Parkinson’s Disease), inflammatory bowel disease (IBD), C…
View article: A Phase 2/3 Study Evaluating the Efficacy and Safety of Pozelimab in Patients with CD55 Deficiency with Hyperactivation of Complement, Angiopathic Thrombosis, and Protein-Losing Enteropathy (CHAPLE Disease)
A Phase 2/3 Study Evaluating the Efficacy and Safety of Pozelimab in Patients with CD55 Deficiency with Hyperactivation of Complement, Angiopathic Thrombosis, and Protein-Losing Enteropathy (CHAPLE Disease) Open
View article: Blau syndrome NOD2 mutations result in loss of NOD2 cross-regulatory function
Blau syndrome NOD2 mutations result in loss of NOD2 cross-regulatory function Open
The studies described here provide an analysis of the pathogenesis of Blau syndrome and thereby the function of NOD2 as seen through the lens of its dysfunction resulting from Blau-associated NOD2 mutations in its nucleotide-binding domain…
View article: Clinical exome sequencing of 1000 families with complex immune phenotypes: Toward comprehensive genomic evaluations
Clinical exome sequencing of 1000 families with complex immune phenotypes: Toward comprehensive genomic evaluations Open
View article: Nodular regenerative hyperplasia in X-linked agammaglobulinemia: An underestimated and severe complication
Nodular regenerative hyperplasia in X-linked agammaglobulinemia: An underestimated and severe complication Open
View article: Robust Antibody and T Cell Responses to SARS-CoV-2 in Patients with Antibody Deficiency
Robust Antibody and T Cell Responses to SARS-CoV-2 in Patients with Antibody Deficiency Open
View article: Recurrent Nodular Regenerative Hyperplasia Following Liver Transplantation in Common Variable Immunodeficiency
Recurrent Nodular Regenerative Hyperplasia Following Liver Transplantation in Common Variable Immunodeficiency Open
Hercun, Julian; Parikh, Esha; Kleiner, David E.; Fuss, Ivan; Uzel, Gulbu; Strober, Warren; Koh, Christopher; Holland, Steven M.; Heller, Theo Author Information
View article: Purification of T Cell Populations
Purification of T Cell Populations Open
This article describes a procedure for isolating T cell subpopulations using various methods including indirect panning and immunopanning by microarray. In these methods, cells are selected by their capacity to bind to antibody‐coated plat…
View article: Bruton tyrosine kinase deficiency augments NLRP3 inflammasome activation and causes IL-1β–mediated colitis
Bruton tyrosine kinase deficiency augments NLRP3 inflammasome activation and causes IL-1β–mediated colitis Open
Bruton tyrosine kinase (BTK) is present in a wide variety of cells and may thus have important non-B cell functions. Here, we explored the function of this kinase in macrophages with studies of its regulation of the NLR family, pyrin domai…
View article: RICK/RIP2 is a NOD2-independent nodal point of gut inflammation
RICK/RIP2 is a NOD2-independent nodal point of gut inflammation Open
Previous studies have shown that inhibition of receptor-interacting serine/threonine kinase (RICK) (also known as RIP2) results in amelioration of experimental colitis. This role has largely been attributed to nucleotide-binding oligomeriz…
View article: The Role of NLRP3 and IL-1β in the Pathogenesis of Inflammatory Bowel Disease
The Role of NLRP3 and IL-1β in the Pathogenesis of Inflammatory Bowel Disease Open
It is logical to assume that a major pro-inflammatory mechanism, i.e., the NLRP3 inflammasome would play a prominent role in the pathogenesis of the Inflammatory Bowel Disease (IBD) in humans. However, while both studies of murine models o…
View article: CVID enteropathy is characterized by exceeding low mucosal IgA levels and interferon-driven inflammation possibly related to the presence of a pathobiont
CVID enteropathy is characterized by exceeding low mucosal IgA levels and interferon-driven inflammation possibly related to the presence of a pathobiont Open
View article: Suppression effect on IFN‑γ of adipose tissue‑derived mesenchymal stem cells isolated from β2‑microglobulin‑deficient mice
Suppression effect on IFN‑γ of adipose tissue‑derived mesenchymal stem cells isolated from β2‑microglobulin‑deficient mice Open
Administration of bone marrow-derived mesenchymal stem cells (MSCs) is a possible treatment for graft-versus-host disease (GVHD) following allogeneic hematopoietic stem cell transplantation and other inflammatory conditions. To address the…
View article: Spleen and Liver Volumetrics as Surrogate Markers of Hepatic Venous Pressure Gradient in Patients With Noncirrhotic Portal Hypertension
Spleen and Liver Volumetrics as Surrogate Markers of Hepatic Venous Pressure Gradient in Patients With Noncirrhotic Portal Hypertension Open
Noncirrhotic portal hypertension (NCPH) is a rare disease that may lead to serious clinical consequences. Currently, noninvasive tools for the assessment of NCPH are absent. We investigated the utility of spleen and liver volumetrics as a …
View article: Loss-of-function CARD8 mutation causes NLRP3 inflammasome activation and Crohn’s disease
Loss-of-function CARD8 mutation causes NLRP3 inflammasome activation and Crohn’s disease Open
In these studies, we evaluated the contribution of the NLRP3 inflammasome to Crohn's disease (CD) in a kindred containing individuals having a missense mutation in CARD8, a protein known to inhibit this inflammasome. Whole exome sequencing…
View article: BACH2 immunodeficiency illustrates an association between super-enhancers and haploinsufficiency
BACH2 immunodeficiency illustrates an association between super-enhancers and haploinsufficiency Open
View article: Tumor growth limited to subcutaneous site vs tumor growth in pulmonary site exhibit differential effects on systemic immunities
Tumor growth limited to subcutaneous site vs tumor growth in pulmonary site exhibit differential effects on systemic immunities Open
To evaluate systemic immunity associated with tumor growth limited to a subcutaneous site versus growth proceeding at multiple tumor sites, we established syngeneic mouse subcutaneous and pulmonary tumor models by local subcutaneous and in…
View article: Akkermansia muciniphila mediates negative effects of IFNγ on glucose metabolism
Akkermansia muciniphila mediates negative effects of IFNγ on glucose metabolism Open
View article: Ataxia telangiectasia associated with nodular regenerative hyperplasia
Ataxia telangiectasia associated with nodular regenerative hyperplasia Open
View article: Experimental Models of Inflammatory Bowel Diseases
Experimental Models of Inflammatory Bowel Diseases Open