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opencc-by-4.0Nov 2022View details →

Autoimmunity in monogenic combined immune deficiencies with associated or syndromic features

Abstract Background: Combined immune deficiencies (CIDs) with associated or syndromic features are a highly heterogeneous subgroup of inherited immune disorders. These patients represent specific clinical complications with an increased risk of autoimmune conditions. Methods: We analyzed data of monogenic patients with syndromic CIDs adopted from the Iranian inborn errors of immunity registry up to January 2022. A comprehensive comparison in terms of demographic, clinical, and immunological features was performed between patients with and without autoimmunity and also among four mutation groups with the most registered cases including ataxia-telangiectasia mutated (ATM), STAT3 AD (LOF) , DNMT3B/ ZBTB24, and WAS mutations. Results: A total of 137 patients with monogenic syndromic CIDs were included. Most commonly mutated genes were the ATM [80 (58.4%)] and STAT3 [19 (13.9%)], followed by DNMT3B [11 (8%)], and WAS [11 (8%)]. More than 18% of all patients with syndromic CIDs, including most DNMT3B / ZBTB24 mutations  patients, were clinically diagnosed with antibody deficiencies before genetic evaluation. Patients with ATM and WAS mutations had the latest age of onset and the lowest age of diagnosis, respectively. Autoimmune disorders were diagnosed in 24 patients at a median age of 3.5 (2.6-6.0) years, 70.6% of which were diagnosed prior to the diagnosis of immunodeficiency. Lymphoproliferation, particularly hepatosplenomegaly, was significantly higher in patients with autoimmunity (p=0.004). Syndromic CID patients with autoimmunity had significantly lower IgG levels. Hematologic autoimmunity mainly immune thrombocytopenic purpura was the most frequent autoimmunity among major groups of ATM, STAT3, DNMT3B/ ZBTB24, and WAS mutations, however ATM-mutated patients present more diversified involved organs including rheumatologic, gastrointestinal and dermatologic autoimmunity. Conclusion: About 18% of patients with monogenic syndromic CIDs developed autoimmunity, mainly in the form of hematological immune diseases. Autoimmunity could be an early-onset involvement with a potential diagnostic impact on suspicious cases of syndromic CIDs.

opencc-by-4.0Jan 2023View details →

Overcoming immune deficiency with allogrooming

Data in excel files formatted for analysis with SPSS.

opencc-by-4.0Feb 2023View details →

Raw Data for the article: Human Amnion-Derived Mesenchymal Stromal/Stem Cells Pre-Conditioning Inhibits Inflammation and Apoptosis of Immune and Parenchymal Cells in an In Vitro Model of Liver Ischemia/Reperfusion

Ischemia/reperfusion injury (IRI) represents one of the leading causes of primary non-function acute liver transplantation failure. IRI, generated by an interruption of organ blood flow and the subsequent restoration upon transplant, i.e., reperfusion, generates the activation of an inflammatory cascade from the resident Kupffer cells, leading first to neutrophils recruitment and second to apoptosis of the parenchyma. Recently, human mesenchymal stromal/stem cells (hMSCs) and derivatives have been implemented for reducing the damage induced by IRI. Interestingly, sparse data in the literature have described the use of human amnion-derived MSCs (hAMSCs) and, more importantly, no evidence regarding hMSCs priming on liver IRI have been described yet. Thus, our study focused on the definition of an in vitro model of liver IRI to test the effect of primed hAMSCs to reduce IRI damage on immune and hepatic cells. We found that the IFNγ pre-treatment and 3D culture of hAMSCs strongly reduced inflammation induced by M1-differentiated macrophages. Furthermore, primed hAMSCs significantly inhibited parenchymal apoptosis at early timepoints of reperfusion by blocking the activation of caspase 3/7. All together, these data demonstrate that hAMSCs priming significantly overcomes IRI effects in vitro by engaging the possibility of defining the molecular pathways involved in this process.

opencc-by-4.0Mar 2023View details →

Accompanying images to the paper NLR immune receptor–nanobody fusions confer plant disease resistance

The following images accompany the paper NLR immune receptor–nanobody fusions confer plant disease resistance, Kourelis J., Marchal C., Posbeyikian A., Harant A., Kamoun S. (2023) Science Vol. 379, No. 6635. <a href="https://doi.org/10.1126/science.abn4116">DOI: 10.1126/science.abn4116</a>   Figure captions: Figure 1. Leaves photographed 12 days after infection with a Potato Virus X variant expressing the fluorescent protein EGFP. Left: Nicotiana benthamiana leaf that doesn't express the pikobody system. Right: Leaf from N. benthamiana line stably expressing the Pikobody system recognizing EGFP.  Figure 2.  Leaves photographed 12 days after infection with a Potato Virus X variant expressing the fluorescent protein mCherry. Both leaves correspond to a N. benthamiana line stably expressing the Pikobody system recognizing EGFP. These results accompany the observations that the Pikobody system targetting EGFP specifically recognizes EGFP, but not mCherry. Figure 3.  Side by side comparison of representative adult N. benthamiana plants, from left to right: wild-type (WT), stably transformed with the rice NLR pair Pikm-1/2 (Pikm) or PikobodyEnhancer (Lines #4 - #1 - #9 - #10).

openJul 2023View details →

Differences in SARS-CoV-2 specific humoral and cellular immune responses after contralateral and ipsilateral COVID-19 vaccination

In an observational study, 303 previously naive individuals were recruited, who received the second dose of the COVID-19 vaccine BNT162b2 on either the ipsilateral (n=147) or the contralateral side (n=156). Spike-specific IgG, IgG-avidity, and neutralizing antibodies were quantified using ELISA and a surrogate assay 2 weeks after dose 2. A subgroup of 143 individuals (64 ipsilateral, 79 contralateral) was analysed for spike-specific CD4 and CD8 T-cells using flow-cytometry.

opennotspecifiedApr 2021View details →

Fig. 2 in Floro-pyrazolo[3,4-d]pyrimidine derivative as a novel plant activator induces two-pathway immune system

Fig. 2. The synthetic route of targeted compounds.

opennotspecifiedApr 2021View details →

Fig. 1 in Floro-pyrazolo[3,4-d]pyrimidine derivative as a novel plant activator induces two-pathway immune system

Fig. 1. Overview of scaffold and derivatives design.

opennotspecifiedApr 2021View details →

Fig. 3 in Floro-pyrazolo[3,4-d]pyrimidine derivative as a novel plant activator induces two-pathway immune system

Fig. 3. Design of target compounds.

opencc-by-4.0Oct 2023View details →

Identification of breast cancer subgroups and immune characterization based on glutamine metabolism-related genes

Open the record for dataset details and reuse information.

controlledIPD-YESFeb 2026View details →

CHOICE KERGUELEN 2 : Consequences of Longterm Confinement on Immunity in the Sub-Antarctic Islands : Follow-up of Volunteers on the Kerguelen Islands

ClinicalTrials.gov study NCT07275489. IPD Sharing: YES. Countries: 1. Publications: 0.

Study of Vitespen (HSPPC-96, Oncophage ®) for Immune Response Assessment in Participants With Resectable Renal Cell Carcinoma at Intermediate Risk of Recurrence

ClinicalTrials.gov study NCT01147536. IPD Sharing: Not stated. Countries: 2. Publications: 0.

A Comparison of Fluconazole and Ketoconazole in the Treatment of Fungal Infections of the Throat in Patients With Weakened Immune Systems

ClinicalTrials.gov study NCT00002304. IPD Sharing: Not stated. Countries: 2. Publications: 1.

Generation of Anti-HCV Antibodies From Bone Marrow: Defining the Repertoire of Immune Response to HCV Quasispecies

ClinicalTrials.gov study NCT00004851. IPD Sharing: Not stated. Countries: 1. Publications: 3.

controlledIPD-YESFeb 2026View details →

Safety and Immune Response Study of GSK Biologicals' Influenza Virus Vaccine 1388442A Compared With Fluarix

ClinicalTrials.gov study NCT00693706. IPD Sharing: YES. Countries: 1. Publications: 0.

An Open-label Safety Study of Lusutrombopag (S-888711) in Adults With Chronic Immune Thrombocytopenia (ITP)

ClinicalTrials.gov study NCT01129024. IPD Sharing: Not stated. Countries: 1. Publications: 0.

Mechanisms of Immune Reconstitution & Reduced Immune Activation Following Darunavir-based ART

ClinicalTrials.gov study NCT01869634. IPD Sharing: Not stated. Countries: 1. Publications: 0.

A Controlled Trial to Evaluate the Effects of GanedenBC30 on the Immune System

ClinicalTrials.gov study NCT01184456. IPD Sharing: Not stated. Countries: 1. Publications: 0.

The Effects of Upper Airway and Digestive Tract Tumors on the Immune System

ClinicalTrials.gov study NCT00001603. IPD Sharing: Not stated. Countries: 1. Publications: 3.

Immune System Modulation and Outcome in High-risk cSCC Treated With Surgery and Radiotherapy

ClinicalTrials.gov study NCT05246228. IPD Sharing: UNDECIDED. Countries: 0. Publications: 17.

Human Immune Globulin in Treating Patients With Primary Amyloidosis That is Causing Heart Dysfunction

ClinicalTrials.gov study NCT00547365. IPD Sharing: Not stated. Countries: 1. Publications: 0.