China
Africa
Explore chapters and articles related to this topic
Benign Disorders of Leukocytes
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Gene L. Gulati, Zoran Gatalica, Bong H. Hyun
Leukocyte adhesion protein deficiency (LAD) is a rare autosomal recessive disorder of leukocyte adhesion and chemotaxis. Leukocytes from patients with LAD adhere poorly to endothelial cells and exhibit defective margination and chemotaxis, leading to recurrent severe infections. Leukocyte adhesion deficiency 1 (LAD1) is caused by defects in CD18 gene, which codes for the common beta-2 subunit of the leukocyte integrins LFA-1 (CD11a/CD18), Mac-1 (CD11b/CD18), and pl50,95 (CD11c/CD18). Patients with LAD2 exhibit normal expression of CD18 but lack sialyl Lewis X antigen (selectin ligand) and similarly do not adhere to endothelial cells. Neutrophil adherence and aggregation deficits in general population are most commonly drug induced (glucocorticoids, alcohol, etc.), or acquired (hemodialysis, myelodysplastic syndromes, etc.). Patients with LAD have persistent leukocytosis (15–20 × 109/fL), but the leukocytes appear morphologically normal. The laboratory diagnosis of LAD is generally made by flow cytometric analysis of blood neutrophils using antibodies specific for CD11 or CD18 antigens.
Dexmedetomidine modulates mitochondrial dynamics to protect against endotoxin-induced lung injury via the protein kinase C-ɑ/haem oxygenase-1 signalling pathway
Published in Biomarkers, 2022
Kai Song, Jia Shi, Lina Zhan, Qiaoying Gao, Jing Yang, Shuan Dong, Yuan Zhang, Jianbo Yu
It was found that there was an increase of plasma levels of TNF-a, IL-6 and IL-1β in group L, LAD1, LAD2 and LDP when compared with those in group C (p < 0.01 for all), indicating an inducing effect of LPS on lung inflammation. In contrast, administration of DEX reduced these inflammatory cytokine levels under LPS condition (p < 0.01 for all; LAD1 OR LAD2 versus L). However, the anti-inflammatory function of reducing levels of TNF-a, IL-6 and IL-1β was suppressed via PKC-ɑ inhibitor CHE (p < 0.01 for all; LDP versus LAD1 OR LAD2). (Figure 3(A–C))
Deficiency of autoimmune regulator impairs the immune tolerance effect of bone marrow-derived dendritic cells in mice
Published in Autoimmunity, 2018
Feifei Huo, Dongbei Li, Bo Zhao, Yadong Luo, Bingjie Zhao, Xueyang Zou, Yi Li, Wei Yang
In the central immune system, Aire functions to eliminate autoreactive T cells by regulating TRAs expression in mTECs and DCs and thereby maintaining immune tolerance [15,16]. Therefore, in order to investigate the effect of Aire on TRAs expression in BMDCs, RT-qPCR was employed to analyze the expression of several autoimmune disease-related TRAs in BMDCs, including type 1 diabetes-associated TRAs (insulin II (Ins2), glutamic acid decarboxylase (GAD) 65/67, insulinoma antigen-2 (IA-2), islet-specific glucose-6-phosphatase catalytic subunit-related protein (IGRP), and Ras-related associated with diabetes (RRAD)), an uveitis-associated TRA (retinol-binding protein 3 (Rbp3)), a Sjögren's syndrome-associated TRA (salivary protein 1 (Spt1)), hypothyroidism-associated TRAs (NACHT, LRR, and PYD domains-containing protein 5 (Nalp5)), an experimental autoimmune encephalomyelitis (EAE)/multiple sclerosis (MS)-associated TRA (myelin oligodendrocyte glycoprotein (Mog)), a Graves’ disease-associated TRA (major urinary protein-1(MUP1)), a pemphigus foliaceus-associated TRA (desmoglein-1-alpha (Dsg1a)), a linear IgA dermatitis-associated TRA (leukocyte adhesion deficiency-1 (Lad1)), and an autoimmune gastritis-associated TRA (ATPase H+/K + transporting alpha subunit (Atp4a)). Spectrin alpha 2 (Spna2) was used as the negative control. Although Spna2 is related to Sjögren's syndrome, its expression is independent of Aire. The results showed that the Ins2, GAD65/67, IA-2, IGRP, Rbp3, Spt1, Nalp5, Mog, and MUP1 expression levels were significantly lower in the KO-BMDCs than in the WT-BMDCs. Additionally, the KO-BMDCs did not express Lad1. And the Dsg1a expression was not detected both in BMDCs and positive control which may be due to its low expression in these tissue and cells or the nonspecific primers we used (Figure 2 and Supplementary Figure 1). These results demonstrate that ectopic expression of Aire in BMDCs promotes the expression of a number of TRA. Thus, Aire may also induce immune tolerance by modulating TRAs expression in BMDCs. However, the precise underlying mechanisms need further investigation.