Dendritic cells and monocyte subsets in children with Gaucher disease

Zahran, Asmaa M; Saad, Khaled; Abdallah, Alameldin M.; Gad, Eman F.; Abdel-Raheem, Yasser F.; Zahran, Zeinab Albadry M.; Abdelsalam, Eman M. Nagiub; Elhoufey, Amira; Alruwaili, Thamer A.M.; Mahmoud, Khaled Hashim; Elsayh, Khalid I

doi:10.1038/s41390-020-01300-w

Cited by 2 publications

(4 citation statements)

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“…The same results were obtained previously 11 , 28 , 29 . This decrease in NK cells may be due to continuous stimulation and chronic apoptosis and may be a risk factor for the B-cell malignancy and other lymphoid tumors previously reported in GD patients 1 , 29 . On the other hand, Limgala et al 24 observed no significant difference in the percentage of NK cells in GD patients under ERT versus healthy controls.…”

Section: Discussionmentioning

confidence: 73%

“…Gaucher disease (GD) is an inherited lysosomal storage disease with a defect in the GBA1 gene, resulting in an abnormal configuration and function of β-glucocerebrosidase 1 . The role of this enzyme is to break down glucosylceramide into glucose and ceramide; thus, the consequence of its defect is the buildup of glucosylceramide in the lysosomes of the monocyte/macrophage line of the reticuloendothelial system, resulting in the evolution of these cells into Gaucher cells, which boast a crumpled tissue paper appearance 1 – 3 . Three clinical forms of GD have been defined according to the existence or absence of any neurological manifestation 2 , 4 .…”

Section: Introductionmentioning

confidence: 99%

“…The complex pathophysiology of GD could be due to not merely the loading of monocyte and macrophage lysosomes with undegraded glucosylceramide; in addition, there are also remarkable immunological irregularities that might be the result of an interruption in the normal immunological role of lysosomes as well as the effect of accumulated glucosylceramide 1 . In GD, the previous research showed ongoing stimulation of the immune system with excessive release of proinflammatory cytokines as interleukin-1 (IL-1) and its receptor antagonists, IL-2, -6, -8, -10, and 18, as well as tumor necrosis factor, transforming growth factor, and macrophage colony-stimulating factor from the macrophage/monocyte lineage 5 .…”

Section: Introductionmentioning

confidence: 99%

“…Two types of T-cells exist helper CD4 T-cells and cytotoxic CD8 T-cells. Cytotoxic CD8 cells have a significant role in the host's immune defense against intracellular pathogens, viruses, and tumors through the production of lytic substances 1 . They can be categorized based on their cytokine profile into multiple subsets, such as T cytotoxic 1 (Tc1), T cytotoxic 2 (Tc2), T cytotoxic 9 (Tc9), T cytotoxic 17 (Tc17), and CD8 T regulatory cells 7 .…”

Section: Introductionmentioning

confidence: 99%

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Upregulation of Cytotoxic T-cells in pediatric patients with Gaucher disease

Zahran

Saad

Elsayh

et al. 2022

Sci Rep

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Cytotoxic (CD8) T-cells and natural killer (NK) cells have a significant immune function role. The ongoing stimulation of immunity and the excessive release of proinflammatory cytokines observed in pediatric patients with Gaucher disease (GD) can affect immune cells. Few studies have looked at the proportion of cytotoxic CD8 T-cells and their subsets in children with GD. A prospective case–control study was performed involving twenty pediatric patients with type 1 GD and twenty healthy age-matched controls. All patients received regular enzyme replacement therapy (ERT) for at least 6 months before the study. Complete blood count and flow cytometric analyses of CD8 T, Tc1, Tc2, NK, and NK T-cells were performed. GD patients showed significantly increased of CD8 T, Tc1 and significantly decreased NK cells frequencies when compared to healthy controls. However, no significant difference in Tc2 and NK T-cells was found between the studied groups. GD patients on regular ERT have increased CD8+ T-cell frequencies, predominantly Tc1, together with a reduction in NK cells than in healthy controls. These crucial immunological changes may contribute to some extent to the pathogenesis and the progression of GD.

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Section: Discussionmentioning

confidence: 73%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Upregulation of Cytotoxic T-cells in pediatric patients with Gaucher disease

Zahran

Saad

Elsayh

et al. 2022

Sci Rep

Self Cite

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Targeting the Complement–Sphingolipid System in COVID-19 and Gaucher Diseases: Evidence for a New Treatment Strategy

Trivedi

Magnusen

Rani

et al. 2022

IJMS

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Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2)-induced disease (COVID-19) and Gaucher disease (GD) exhibit upregulation of complement 5a (C5a) and its C5aR1 receptor, and excess synthesis of glycosphingolipids that lead to increased infiltration and activation of innate and adaptive immune cells, resulting in massive generation of pro-inflammatory cytokines, chemokines and growth factors. This C5a–C5aR1–glycosphingolipid pathway- induced pro-inflammatory environment causes the tissue damage in COVID-19 and GD. Strikingly, pharmaceutically targeting the C5a–C5aR1 axis or the glycosphingolipid synthesis pathway led to a reduction in glycosphingolipid synthesis and innate and adaptive immune inflammation, and protection from the tissue destruction in both COVID-19 and GD. These results reveal a common involvement of the complement and glycosphingolipid systems driving immune inflammation and tissue damage in COVID-19 and GD, respectively. It is therefore expected that combined targeting of the complement and sphingolipid pathways could ameliorate the tissue destruction, organ failure, and death in patients at high-risk of developing severe cases of COVID-19.

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Dendritic cells and monocyte subsets in children with Gaucher disease

Cited by 2 publications

References 21 publications

Upregulation of Cytotoxic T-cells in pediatric patients with Gaucher disease

Upregulation of Cytotoxic T-cells in pediatric patients with Gaucher disease

Targeting the Complement–Sphingolipid System in COVID-19 and Gaucher Diseases: Evidence for a New Treatment Strategy

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