ADAMTS13 deficiency and immunological abnormalities in patients with systemic sclerosis

Gerlicz-Kowalczuk, Zofia; Torzecka, Jolanta Dorota; Dziankowska-Zaborszczyk, Elżbieta; Ograczyk, Alicja; Zalewska-Janowska, Anna; Woznicka, Anna K; Dziankowska‐Bartkowiak, Bożena

doi:10.5114/ada.2018.75240

Cited by 2 publications

(1 citation statement)

References 11 publications

(10 reference statements)

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“…ADAMTS13 was lower in lcSSc without ILD compared to healthy controls in our plasma samples, which is in keeping with previous findings of SSc serum 23 . ADAMTS13, which cleaves unusually large (and hyperreactive) von Willebrand factor (ULVWF) released from activated endothelial cells to smaller and less active forms, is a metalloproteinase involved in von Willebrand factor coagulation and platelet activation pathways, and is possibly related to the vasculopathy and endotheliopathy present in SSc 23 , 24 .…”

Section: Discussionmentioning

confidence: 99%

Circulating cytokine levels in systemic sclerosis related interstitial lung disease and idiopathic pulmonary fibrosis

Zheng

Keen

Fritzler

et al. 2023

Sci Rep

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Exploration of cytokine levels in systemic sclerosis-associated interstitial lung disease (SSc-ILD) and idiopathic pulmonary fibrosis (IPF) is needed to find common and diverse biomolecular pathways. Circulating levels of 87 cytokines were compared amongst 19 healthy controls and consecutive patients with SSc-ILD (n = 39), SSc without ILD (n = 29), and IPF (n = 17) recruited from a Canadian centre using a log-linear model adjusted for age, sex, baseline forced vital capacity (FVC), and immunosuppressive or anti-fibrotic treatment at time of sampling. Also examined was annualized change in FVC. Four cytokines had Holm’s corrected p-values less than 0.05. Eotaxin-1 levels were increased approximately two-fold in all patient categories compared to healthy controls. Interleukin-6 levels were eight-fold higher in all ILD categories compared to healthy controls. MIG/CXCL9 levels increased two-fold more in all but one patient category compared to healthy controls. Levels of a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13, (ADAMTS13) were lower for all categories of patients compared to controls. No substantial association was found for any of the cytokines with FVC change. Observed cytokine differences suggest both common and diverse pathways leading to pulmonary fibrosis. Further studies evaluating longitudinal change of these molecules would be informative.

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Section: Discussionmentioning

confidence: 99%

Circulating cytokine levels in systemic sclerosis related interstitial lung disease and idiopathic pulmonary fibrosis

Zheng

Keen

Fritzler

et al. 2023

Sci Rep

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Determination of molecular pathways and gene ontology of genes associated with Raynaud’s phenomenon

Öztan

2024

Turkish Journal of Biochemistry

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Objectives Raynaud’s phenomenon (RF) is a disease that causes discoloration of the fingers. The purpose of this study is to identify the molecular pathways in which genes related to RP illness are involved, as well as uncover the biological processes and molecular functions connected with those genes via the use of gene ontology (GO) analysis. Methods Genes associated with RP in the MalaCards Human Diseases database were detected. Twenty genes obtained from the MalaCards Human Diseases database were included in the study for gene ontology analysis via the STRING database. Accordingly, possible interactions between 20 genes were determined through STRING and network enrichment was performed. Results A significant enrichment by gene ontology enrichment analysis was detected in a subset of genes involved in biological processes including cellular response to luteinizing hormone stimulus, negative regulation of fibrinolysis, negative regulation of smooth muscle cell apoptotic process, plasminogen activation, cellular response to follicle-stimulating hormone stimulus. The assay for molecular function determined enrichment of a subset of genes in chemoattractant activity, growth factor activity, heparin binding, sulfur compound binding, growth factor receptor binding. Through the use of KEGG pathways, we were able to identify many molecular processes that contribute to RP, including the AGE-RAGE signaling pathway in diabetic complications, complement and coagulation cascades, fluid shear stress, atherosclerosis. Conclusions Some individuals may have a genetic predisposition to the onset of Raynaud’s phenomenon. Our data showed that it is associated with genes involved in vascular damage and fibrosis, especially in RP. Therefore, we can include RP disease in the group of vascular diseases.

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ADAMTS13 deficiency and immunological abnormalities in patients with systemic sclerosis

Cited by 2 publications

References 11 publications

Circulating cytokine levels in systemic sclerosis related interstitial lung disease and idiopathic pulmonary fibrosis

Circulating cytokine levels in systemic sclerosis related interstitial lung disease and idiopathic pulmonary fibrosis

Determination of molecular pathways and gene ontology of genes associated with Raynaud’s phenomenon

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