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Article ; Online: Integrated omics analysis for characterization of the contribution of high fructose corn syrup to non-alcoholic fatty liver disease in obesity.

Papadopoulos, Grigorios / Legaki, Aigli-Ioanna / Georgila, Konstantina / Vorkas, Panagiotis / Giannousi, Eirini / Stamatakis, George / Moustakas, Ioannis I / Petrocheilou, Maria / Pyrina, Iryna / Gercken, Bettina / Kassi, Eva / Chavakis, Triantafyllos / Pateras, Ioannis S / Panayotou, George / Gika, Helen / Samiotaki, Martina / Eliopoulos, Aristides G / Chatzigeorgiou, Antonios

Metabolism: clinical and experimental

2023 Volume 144, Page(s) 155552

Abstract: Background: High-Fructose Corn Syrup (HFCS), a sweetener rich in glucose and fructose, is nowadays widely used in beverages and processed foods; its consumption has been correlated to the emergence and progression of Non-Alcoholic Fatty Liver Disease ( ... ...

Abstract	Background: High-Fructose Corn Syrup (HFCS), a sweetener rich in glucose and fructose, is nowadays widely used in beverages and processed foods; its consumption has been correlated to the emergence and progression of Non-Alcoholic Fatty Liver Disease (NAFLD). Nevertheless, the molecular mechanisms by which HFCS impacts hepatic metabolism remain scarce, especially in the context of obesity. Besides, the majority of current studies focuses either on the detrimental role of fructose in hepatic steatosis or compare separately the additive impact of fructose versus glucose in high fat diet-induced NAFLD. Aim: By engaging combined omics approaches, we sought to characterize the role of HFCS in obesity-associated NAFLD and reveal molecular processes, which mediate the exaggeration of steatosis under these conditions. Methods: Herein, C57BL/6 mice were fed a normal-fat-diet (ND), a high-fat-diet (HFD) or a HFD supplemented with HFCS (HFD-HFCS) and upon examination of their metabolic and NAFLD phenotype, proteomic, lipidomic and metabolomic analyses were conducted to identify HFCS-related molecular alterations of the hepatic metabolic landscape in obesity. Results: Although HFD and HFD-HFCS mice displayed comparable obesity, HFD-HFCS mice showed aggravation of hepatic steatosis, as analysis of the lipid droplet area in liver sections revealed (12,15 % of total section area in HFD vs 22,35 % in HFD-HFCS), increased NAFLD activity score (3,29 in HFD vs 4,86 in HFD-HFCS) and deteriorated hepatic insulin resistance, as compared to the HFD mice. Besides, the hepatic proteome of HFD-HFCS mice was characterized by a marked upregulation of 5 core proteins implicated in de novo lipogenesis (DNL), while an increased phosphatidyl-cholines(PC)/phosphatidyl-ethanolamines(PE) ratio (2.01 in HFD vs 3.04 in HFD-HFCS) was observed in the livers of HFD-HFCS versus HFD mice. Integrated analysis of the omics datasets indicated that Tricarboxylic Acid (TCA) cycle overactivation is likely contributing towards the intensification of steatosis during HFD-HFCS-induced NAFLD. Conclusion: Our results imply that HFCS significantly contributes to steatosis aggravation during obesity-related NAFLD, likely deriving from DNL upregulation, accompanied by TCA cycle overactivation and deteriorated hepatic insulin resistance.
MeSH term(s)	Mice ; Animals ; Non-alcoholic Fatty Liver Disease/genetics ; Non-alcoholic Fatty Liver Disease/metabolism ; High Fructose Corn Syrup/adverse effects ; High Fructose Corn Syrup/metabolism ; Insulin Resistance/genetics ; Proteomics ; Mice, Inbred C57BL ; Liver/metabolism ; Obesity/genetics ; Obesity/metabolism ; Fructose/adverse effects ; Fructose/metabolism ; Glucose/metabolism ; Diet, High-Fat/adverse effects
Chemical Substances	High Fructose Corn Syrup ; Fructose (30237-26-4) ; Glucose (IY9XDZ35W2)
Language	English
Publishing date	2023-03-28
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	80230-x
ISSN	1532-8600 ; 0026-0495
ISSN (online)	1532-8600
ISSN	0026-0495
DOI	10.1016/j.metabol.2023.155552
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Article ; Online: Comparison of Long-term Ambulatory Function in Patients with Duchenne Muscular Dystrophy Treated with Eteplirsen and Matched Natural History Controls.

Mendell, Jerry R / Khan, Navid / Sha, Nanshi / Eliopoulos, Helen / McDonald, Craig M / Goemans, Nathalie / Mercuri, Eugenio / Lowes, Linda P / Alfano, Lindsay N

Journal of neuromuscular diseases

2021 Volume 8, Issue 4, Page(s) 469–479

Abstract: Background: Duchenne muscular dystrophy (DMD) is a rare, X-linked, fatal, degenerative neuromuscular disease caused by DMD gene mutations. A relationship between exon skipping and dystrophin production in exon 51-amenable patients treated with ... ...

Abstract	Background: Duchenne muscular dystrophy (DMD) is a rare, X-linked, fatal, degenerative neuromuscular disease caused by DMD gene mutations. A relationship between exon skipping and dystrophin production in exon 51-amenable patients treated with eteplirsen (EXONDYS 51®) is established. Once-weekly eteplirsen significantly increased dystrophin, with slower decline in ambulatory function compared to baseline. Long-term treatment with eteplirsen leads to accumulation of dystrophin over time and observed functional benefits in patients with DMD. Objective: Compare long-term ambulatory function in eteplirsen-treated patients versus controls. Methods: Study 201/202 included 12 eteplirsen-treated patients assessed twice/year for ambulatory function over 4 years. Ambulatory evaluations (6-minute walk test [6MWT], loss of ambulation, and North Star Ambulatory Assessment [NSAA]) were compared with matched controls from Italian Telethon and Leuven registries. Results: At Years 3 and 4, eteplirsen-treated patients demonstrated markedly greater mean 6MWT than controls (difference in change from baseline of 132 m [95%CI (29, 235), p = 0.015] at Year 3 and 159 m [95%CI (66, 253), p = 0.002] at Year 4). At Year 4, a significantly greater proportion of eteplirsen-treated patients were still ambulant versus controls (10/12 vs 3/11; p = 0.020). At Year 3, eteplirsen-treated patients demonstrated milder NSAA decline versus controls (difference in change from baseline of 2.6, 95%CI [-6, 11]), however, the difference was not statistically significant; Year 4 control NSAA data were not available. Conclusion: In this retrospective matched control study, eteplirsen treatment resulted in attenuation of ambulatory decline over a 4-year observation period, supporting long-term benefit in patients with DMD.
MeSH term(s)	Adolescent ; Case-Control Studies ; Child ; Dystrophin/drug effects ; Exons ; Humans ; Male ; Morpholinos/therapeutic use ; Muscular Dystrophy, Duchenne/drug therapy ; Mutation ; Retrospective Studies ; Walk Test ; Walking
Chemical Substances	DMD protein, human ; Dystrophin ; Morpholinos ; eteplirsen (AIW6036FAS)
Language	English
Publishing date	2021-01-30
Publishing country	Netherlands
Document type	Journal Article
ISSN	2214-3602
ISSN (online)	2214-3602
DOI	10.3233/JND-200548
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Article ; Online: Immunoglobulin and B-cell disturbances in patients with chronic idiopathic neutropenia.

Mavroudi, Irene / Eliopoulos, Aristides G / Pontikoglou, Charalampos / Pyrovolaki, Katerina / Damianaki, Athina / Koutala, Helen / Zervou, Maria I / Ximeri, Maria / Mastrodemou, Semeli / Kanellou, Peggy / Goulielmos, George N / Papadaki, Helen A

Clinical immunology (Orlando, Fla.)

2017 Volume 183, Page(s) 75–81

Abstract: Chronic idiopathic neutropenia (CIN) is a granulocytic disorder associated with presence of activated, myelosuppressive T-lymphocytes. In the present study we have evaluated constituents of humoral immunity in CIN patients (n=48) compared to healthy ... ...

Abstract	Chronic idiopathic neutropenia (CIN) is a granulocytic disorder associated with presence of activated, myelosuppressive T-lymphocytes. In the present study we have evaluated constituents of humoral immunity in CIN patients (n=48) compared to healthy controls (n=52). CIN patients displayed lower serum IgG levels due to a reduction in IgG1, IgG3, IgG4 but not IgG2, lower IgA and increased IgM levels compared to controls. The proportion of CD19
Language	English
Publishing date	2017-10
Publishing country	United States
Document type	Journal Article
ZDB-ID	1459903-x
ISSN	1521-7035 ; 1521-6616
ISSN (online)	1521-7035
ISSN	1521-6616
DOI	10.1016/j.clim.2017.07.009
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Article ; Online: Eteplirsen Treatment Attenuates Respiratory Decline in Ambulatory and Non-Ambulatory Patients with Duchenne Muscular Dystrophy.

Khan, Navid / Eliopoulos, Helen / Han, Lixin / Kinane, T Bernard / Lowes, Linda P / Mendell, Jerry R / Gordish-Dressman, Heather / Henricson, Erik K / McDonald, Craig M

Journal of neuromuscular diseases

2019 Volume 6, Issue 2, Page(s) 213–225

Abstract: Background: Duchenne muscular dystrophy (DMD) patients experience skeletal muscle degeneration, including respiratory muscles. Respiratory decline in glucocorticoid-treated DMD patients, measured by percent predicted forced vital capacity (FVC% p), is ... ...

Abstract	Background: Duchenne muscular dystrophy (DMD) patients experience skeletal muscle degeneration, including respiratory muscles. Respiratory decline in glucocorticoid-treated DMD patients, measured by percent predicted forced vital capacity (FVC% p), is typically 5% annually in patients aged 10 to 18 years. Objective: Evaluate the effects of eteplirsen on FVC% p annual change in 3 trials versus matched Cooperative International Neuromuscular Research Group Duchenne Natural History Study (CINRG DNHS) controls. Methods: Eteplirsen studies 201/202 evaluated eligible ambulatory DMD patients for at least 4 years, study 204 evaluated primarily non-ambulatory DMD patients for 2 years, and ongoing study 301 is evaluating ambulatory DMD patients for 2 years (interim analysis is included). Eteplirsen-treated patients (n = 74) were amenable to exon 51 skipping and were receiving glucocorticoids. Three CINRG DNHS cohorts included: glucocorticoid-treated patients amenable to exon 51 skipping (Exon 51 CINRG DNHS; n = 20), all glucocorticoid-treated CINRG patients (All CINRG DNHS; n = 172), and all glucocorticoid-treated genotyped CINRG DNHS patients (Genotyped CINRG DNHS; n = 148). FVC% p assessments between ages 10 and <18 years were included for all patients; mixed-model analyses characterized FVC% p annual change. Results: FVC% p annual change was greater for CINRG DNHS Exon 51 controls (- 6.00) versus patients in studies 201/202, study 204, and study 301 (- 2.19, P < 0.001; - 3.66, P 0.004; and - 3.79, P 0.017, respectively). FVC% p annual change in all eteplirsen studies suggested treatment benefit compared with the Genotyped CINRG DNHS (- 5.67) and All CINRG DNHS (- 5.56) cohorts (P < 0.05, all comparisons). Conclusions: Significant, clinically meaningful attenuation of FVC%p decline was observed in eteplirsen-treated patients versus CINRG DNHS controls.
MeSH term(s)	Adolescent ; Child ; Clinical Trials as Topic ; Humans ; Male ; Morpholinos/therapeutic use ; Muscular Dystrophy, Duchenne/drug therapy ; Respiration/drug effects ; Vital Capacity/drug effects
Chemical Substances	Morpholinos ; eteplirsen (AIW6036FAS)
Language	English
Publishing date	2019-03-11
Publishing country	Netherlands
Document type	Journal Article
ISSN	2214-3602
ISSN (online)	2214-3602
DOI	10.3233/JND-180351
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Article: The role of apoptosis in the pathophysiology of chronic neutropenias associated with bone marrow failure.

Papadaki, Helen A / Eliopoulos, George D

Cell cycle (Georgetown, Tex.)

2003 Volume 2, Issue 5, Page(s) 447–451

Abstract: Chronic neutropenia syndromes associated with bone marrow (BM) failure comprise distinct congenital and acquired hematologic disorders with varying degree of neutropenia due to decreased or ineffective BM neutrophil production. Recent evidence suggests ... ...

Abstract	Chronic neutropenia syndromes associated with bone marrow (BM) failure comprise distinct congenital and acquired hematologic disorders with varying degree of neutropenia due to decreased or ineffective BM neutrophil production. Recent evidence suggests that defective granulocytopoiesis in these neutropenia states is a consequence of accelerated apoptotic cell death of BM myeloid progenitor cells and/or their differentiated progeny. Inherited or spontaneously appearing mutations in the ELA2 gene encoding for neutrophil elastase have been implicated in the accelerated apoptotic process of the BM myeloid cells in patients with cyclic and severe congenital neutropenia. A disturbed balance between pro-apoptotic and anti-apoptotic intracellular or membrane molecules such as downregulation of the bcl-2 family members or upregulation of the death receptor Fas, have been implicated in neutropenia associated with myelokathexis, Shwachman-Diamond syndrome and acquired chronic idiopathic neutropenia of adult. In this review we summarize the available evidence suggesting that abnormally increased apoptosis and impaired proliferative and differentiating properties of neutrophil progenitor and precursor cells represent a common pathogenetic mechanism for impaired granulocytopoiesis in both acquired idiopathic and congenital neutropenia states. The underlying distinct cellular and molecular abnormalities and the role of the BM microenvironment are extensively analysed.
MeSH term(s)	Apoptosis/physiology ; Bone Marrow Cells/metabolism ; Bone Marrow Cells/pathology ; DNA-Binding Proteins/genetics ; Granulocytes/metabolism ; Granulocytes/pathology ; Humans ; Leukocyte Elastase/metabolism ; Mutation ; Neutropenia/genetics ; Neutropenia/physiopathology ; Neutrophils/metabolism ; Neutrophils/pathology ; Proteins/genetics ; Proto-Oncogene Proteins c-bcl-2/metabolism ; Stem Cells/cytology ; Stem Cells/metabolism ; Transcription Factors ; Wiskott-Aldrich Syndrome Protein
Chemical Substances	DNA-Binding Proteins ; GFI1 protein, human ; Proteins ; Proto-Oncogene Proteins c-bcl-2 ; Transcription Factors ; WAS protein, human ; Wiskott-Aldrich Syndrome Protein ; Leukocyte Elastase (EC 3.4.21.37)
Language	English
Publishing date	2003-09-05
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2146183-1
ISSN	1551-4005 ; 1538-4101 ; 1554-8627
ISSN (online)	1551-4005
ISSN	1538-4101 ; 1554-8627
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Zs.A 5881: Show issues

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Article: Helicobacter pylori infection is probably the cause of chronic idiopathic neutropenia (CIN)-associated splenomegaly.

Papadaki, Helen A / Pontikoglou, Charis / Eliopoulos, Dimitris G / Pyrovolaki, Katerina / Spyridaki, Rania / Eliopoulos, George D

American journal of hematology

2006 Volume 81, Issue 2, Page(s) 142–144

Abstract: Splenic volume and Helicobacter pylori (H. pylori) infection were evaluated in 67 patients with chronic idiopathic neutropenia (CIN) and 39 healthy individuals. Using ultrasound, splenomegaly was found in 61.7% of H. pylori-infected subjects compared to ... ...

Abstract	Splenic volume and Helicobacter pylori (H. pylori) infection were evaluated in 67 patients with chronic idiopathic neutropenia (CIN) and 39 healthy individuals. Using ultrasound, splenomegaly was found in 61.7% of H. pylori-infected subjects compared to only 8.7% noted in the group of H. pylori-non-infected individuals (P < 0.0001). Splenomegaly was also found in 47.8% of CIN patients compared to 12.8% in the group of non-CIN subjects (P = 0.0003). However, applying the two-way ANOVA test, a statistically significant effect on splenic volume was documented for "factor H. pylori " (F1(102) = 16.800, P < 0.0001) but not for "factor CIN" (F1(102) = 3.213, P = 0.0760), suggesting that CIN-associated splenomegaly is probably due to H. pylori infection.
MeSH term(s)	Adult ; Aged ; Analysis of Variance ; Chronic Disease ; Female ; Helicobacter Infections/complications ; Helicobacter pylori ; Humans ; Male ; Middle Aged ; Neutropenia/complications ; Splenomegaly/diagnostic imaging ; Splenomegaly/etiology ; Ultrasonography
Language	English
Publishing date	2006-02
Publishing country	United States
Document type	Journal Article
ZDB-ID	196767-8
ISSN	1096-8652 ; 0361-8609
ISSN (online)	1096-8652
ISSN	0361-8609
DOI	10.1002/ajh.20496
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Zs.A 1251: Show issues

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Article ; Online: The CD40/CD40 ligand interactions exert pleiotropic effects on bone marrow granulopoiesis.

Mavroudi, Irene / Papadaki, Vassiliki / Pyrovolaki, Katerina / Katonis, Pavlos / Eliopoulos, Aristides G / Papadaki, Helen A

Journal of leukocyte biology

2011 Volume 89, Issue 5, Page(s) 771–783

Abstract: CD40 is a member of the TNFR family and upon interaction with its cognate ligand (CD40L), induces diverse biologic responses related to cell survival/growth. As altered CD40/CD40L interactions have been associated with neutropenia, we investigated the ... ...

Abstract	CD40 is a member of the TNFR family and upon interaction with its cognate ligand (CD40L), induces diverse biologic responses related to cell survival/growth. As altered CD40/CD40L interactions have been associated with neutropenia, we investigated the role of CD40/CD40L on human granulopoiesis using immunomagnetically sorted CD34(+), CD34(-)/CD33(+), and CD34(-)/CD33(-)/CD15(+) BM cells, which represent sequential stages of the granulocytic development, the KG-1 cells that constantly express CD34 and CD33, and LTBMCs that mimic the BM microenvironment. CD40 and CD40L were minimally expressed on CD34(+), CD34(-)/CD33(+), and CD34(-)/CD33(-)/CD15(+) cells, but CD40 was substantially induced in the presence of TNF-α. Cross-linking of CD40 in the above cell populations resulted in induction of apoptosis that was enhanced further in the presence of FasL. CD40 activation in primary as wells as in KG-1 cells resulted in Fas up-regulation, providing a mechanism for the CD40-mediated apoptosis. Addition of CD40L in clonogenic assays resulted in a significant decrease in the colony-forming capacity of BMMCs from patients with chronic neutropenia, presumably expressing high levels of CD40 in the progenitor cells, and this effect was reversed upon CD40 blockade. CD40 was constitutively expressed on LTBMC stromal cells and upon activation, resulted in an increase in G-CSF and GM-CSF production. These data show that CD40/CD40L interactions may promote granulopoiesis under steady-state conditions by inducing the stromal release of granulopoiesis-supporting cytokines, whereas under inflammatory conditions, they may affect the granulocytic progenitor/precursor cell survival by accelerating the Fas-mediated apoptosis.
MeSH term(s)	Apoptosis ; Blotting, Western ; Bone Marrow/metabolism ; CD40 Antigens/metabolism ; CD40 Ligand/metabolism ; Cells, Cultured ; Colony-Forming Units Assay ; Cytokines/metabolism ; Flow Cytometry ; Granulocyte Colony-Stimulating Factor/metabolism ; Granulocyte-Macrophage Colony-Stimulating Factor/metabolism ; Granulocytes/metabolism ; Hematopoietic Stem Cells/metabolism ; Humans ; Leukemia, Myeloid, Acute/metabolism ; Leukemia, Myeloid, Acute/pathology ; Neutropenia/metabolism ; Neutropenia/pathology ; Stromal Cells/metabolism
Chemical Substances	CD40 Antigens ; Cytokines ; Granulocyte Colony-Stimulating Factor (143011-72-7) ; CD40 Ligand (147205-72-9) ; Granulocyte-Macrophage Colony-Stimulating Factor (83869-56-1)
Language	English
Publishing date	2011-05
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	605722-6
ISSN	1938-3673 ; 0741-5400
ISSN (online)	1938-3673
ISSN	0741-5400
DOI	10.1189/jlb.0610330
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Zs.A 603: Show issues

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Article ; Online: Long-term treatment with eteplirsen in nonambulatory patients with Duchenne muscular dystrophy.

Medicine

2019 Volume 98, Issue 26, Page(s) e15858

Abstract: This analysis aims to describe the outcomes of two nonambulatory patients with Duchenne muscular dystrophy (DMD) who participated in two clinical studies. The two consecutive trials of eteplirsen (studies 201 and 202) were conducted in patients with DMD ( ...

Abstract	This analysis aims to describe the outcomes of two nonambulatory patients with Duchenne muscular dystrophy (DMD) who participated in two clinical studies. The two consecutive trials of eteplirsen (studies 201 and 202) were conducted in patients with DMD (N = 12) and confirmed genetic mutations amenable to exon 51 skipping.In study 201, 12 patients were randomized to receive once-weekly, double-blind intravenous infusions of eteplirsen 30 or 50 mg/kg or placebo for 24 weeks; patients then received open-label eteplirsen during weeks 25 through 28. All 12 patients continued onto open-label extension study 202 and received long-term treatment with eteplirsen. We compared cardiac, pulmonary, and upper limb function and dystrophin production in the nonambulatory twin patients versus the 10 ambulatory patients through 240 combined treatment weeks.Ten study patients remained ambulatory through both studies, while the identical twin patients both experienced early, rapid loss of ambulation. The twin patients had greater disease severity at baseline (6-minute walk test [6MWT], 330 and 256 m) versus the other patients (n = 10; 6MWT range, 341-418 m). They maintained cardiac and upper limb function through combined week 240, with outcomes similar to those of the patients who remained ambulatory. Dystrophin production was confirmed following eteplirsen treatment.Despite the loss of ambulation, other markers of disease progression remained relatively stable in the eteplirsen-treated twin patients and were similar to those of the ambulatory patients.
MeSH term(s)	Child ; Disease Progression ; Diseases in Twins ; Double-Blind Method ; Dystrophin/genetics ; Dystrophin/metabolism ; Humans ; Male ; Morpholinos/adverse effects ; Morpholinos/therapeutic use ; Muscular Dystrophy, Duchenne/drug therapy ; Muscular Dystrophy, Duchenne/genetics ; Muscular Dystrophy, Duchenne/physiopathology ; RNA Processing, Post-Transcriptional/drug effects ; Severity of Illness Index ; Treatment Outcome ; Walk Test ; Walking
Chemical Substances	DMD protein, human ; Dystrophin ; Morpholinos ; eteplirsen (AIW6036FAS)
Language	English
Publishing date	2019-09-04
Publishing country	United States
Document type	Journal Article ; Randomized Controlled Trial
ZDB-ID	80184-7
ISSN	1536-5964 ; 0025-7974
ISSN (online)	1536-5964
ISSN	0025-7974
DOI	10.1097/MD.0000000000015858
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Article: Increased expression of CD40 on bone marrow CD34+ hematopoietic progenitor cells in patients with systemic lupus erythematosus: contribution to Fas-mediated apoptosis.

Pyrovolaki, Katerina / Mavroudi, Irene / Sidiropoulos, Prodromos / Eliopoulos, Aristides G / Boumpas, Dimitrios T / Papadaki, Helen A

Arthritis and rheumatism

2009 Volume 60, Issue 2, Page(s) 543–552

Abstract: Objective: Patients with systemic lupus erythematosus (SLE) display increased apoptosis of bone marrow (BM) CD34+ hematopoietic progenitor cells. This study was undertaken to evaluate the expression of CD40 and CD40L in the BM of SLE patients, and to ... ...

Abstract	Objective: Patients with systemic lupus erythematosus (SLE) display increased apoptosis of bone marrow (BM) CD34+ hematopoietic progenitor cells. This study was undertaken to evaluate the expression of CD40 and CD40L in the BM of SLE patients, and to explore the possible involvement of these molecules in apoptosis of CD34+ cells. Methods: The proportion and survival characteristics of CD40+ cells within the BM CD34+ fraction from SLE patients and healthy controls were evaluated by flow cytometry. The production of CD40L by BM stromal cells was assessed using long-term BM cultures, and the effect of CD40L on the survival characteristics and clonogenic potential of CD34+ cells was evaluated ex vivo by flow cytometry and clonogenic assays. Results: SLE patients displayed an increased proportion of CD40+ cells within the CD34+ fraction as compared with controls. The CD34+CD40+ subpopulation contained an increased proportion of apoptotic cells compared with the CD34+CD40- fraction in patients and controls, suggesting that CD40 is involved in the apoptosis of CD34+ cells. Stimulation of patients' CD34+ cells with CD40L increased the proportion of apoptotic cells and decreased the proportion of colony-forming cells as compared with untreated cultures. The CD40L-mediated effects were amplified following treatment with recombinant Fas ligand, suggesting that the effects of these ligands are synergistic. CD40L levels were significantly increased in long-term BM culture supernatants and adherent layers of BM cells from SLE patients as compared with controls. Conclusion: These data reveal a novel role for the CD40/CD40L dyad in SLE by demonstrating that up-regulation and induction of CD40 on BM CD34+ cells from patients with SLE contribute to the amplification of Fas-mediated apoptosis of progenitor cells.
MeSH term(s)	Adult ; Aged ; Antigens, CD34/immunology ; Antigens, CD34/metabolism ; Apoptosis/drug effects ; Biomarkers/metabolism ; Bone Marrow Cells/immunology ; Bone Marrow Cells/metabolism ; Bone Marrow Cells/pathology ; CD40 Antigens/immunology ; CD40 Antigens/metabolism ; Cell Survival ; Cells, Cultured ; Clone Cells ; Fas Ligand Protein/pharmacology ; Female ; Health Status ; Hematopoietic Stem Cells/immunology ; Hematopoietic Stem Cells/metabolism ; Hematopoietic Stem Cells/pathology ; Humans ; Lupus Erythematosus, Systemic/immunology ; Lupus Erythematosus, Systemic/metabolism ; Lupus Erythematosus, Systemic/physiopathology ; Male ; Middle Aged ; Severity of Illness Index ; Stromal Cells/immunology ; Stromal Cells/metabolism ; Stromal Cells/pathology ; Young Adult ; fas Receptor/metabolism
Chemical Substances	Antigens, CD34 ; Biomarkers ; CD40 Antigens ; Fas Ligand Protein ; fas Receptor
Language	English
Publishing date	2009-02
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	127294-9
ISSN	1529-0131 ; 0004-3591 ; 2326-5191
ISSN (online)	1529-0131
ISSN	0004-3591 ; 2326-5191
DOI	10.1002/art.24257
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Zs.A 24: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 523: Show issues

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Article: Bone marrow stem cells and stromal cells in autoimmune cytopenias.

Papadaki, Helen A / Marsh, Judith C W / Eliopoulos, George D

Leukemia & lymphoma

2002 Volume 43, Issue 4, Page(s) 753–760

Abstract: High-dose immunosuppression followed by autologous haemopoietic stem cell transplantation (ASCT) is a promising practice for the treatment of severe, resistant autoimmune disorders. Patients with refractory autoimmune cytopenias (AIC), primary or ... ...

Abstract	High-dose immunosuppression followed by autologous haemopoietic stem cell transplantation (ASCT) is a promising practice for the treatment of severe, resistant autoimmune disorders. Patients with refractory autoimmune cytopenias (AIC), primary or secondary to systemic autoimmune diseases (AID) including systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), have been proposed as potential candidates for such a therapeutic procedure. An abnormal immune milieu, however, may affect the number and functional characteristics of stem cells and/or stromal cells in the bone marrow (BM) and might impact on harvesting and engraftment potential of stem cells or on BM reconstitution following engraftment in patients with AIC undergoing ASCT. Using flow cytometry and in vitro culture assays we have shown that patients with primary AIC display increased number of BM CD34+ cells in response to abnormally high production of granulocyte-colony stimulating factor (G-CSF) by BM stroma. In contrast, patients with AIC secondary to systemic AID display increased apoptosis of BM progenitor cells resulting in low CD34+ cell numbers and abnormal haemopoiesis supporting capacity of BM stroma due to the aberrant, local or systemic, inhibitory cytokine production or to intricate interactions between haemopoietic and immune cells present within the BM microenvironment. In this review we summarize the available knowledge on BM stem cell reserve and function and stromal cell function in patients with primary and secondary AIC with special reference to SLE and RA. The underlying mechanisms possibly involved in the pathogenesis of the observed abnormalities are also discussed.
MeSH term(s)	Antigens, CD34/analysis ; Arthritis, Rheumatoid/immunology ; Arthritis, Rheumatoid/physiopathology ; Arthritis, Rheumatoid/therapy ; Bone Marrow Cells/physiology ; Hematopoiesis ; Hematopoietic Stem Cells/physiology ; Humans ; Lupus Erythematosus, Systemic/immunology ; Lupus Erythematosus, Systemic/physiopathology ; Lupus Erythematosus, Systemic/therapy ; Stromal Cells/physiology
Chemical Substances	Antigens, CD34
Language	English
Publishing date	2002-04
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	1042374-6
ISSN	1029-2403 ; 1042-8194
ISSN (online)	1029-2403
ISSN	1042-8194
DOI	10.1080/10428190290016854
Database	MEDical Literature Analysis and Retrieval System OnLINE

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