دورية أكاديمية

Low Plasma Gelsolin Concentrations in Chronic Granulomatous Disease.

التفاصيل البيبلوغرافية
العنوان: Low Plasma Gelsolin Concentrations in Chronic Granulomatous Disease.
المؤلفون: Audley J; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Gliniewicz EF; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Zarember KA; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Hong HS; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Wald G; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Kuhns DB; Neutrophil Monitoring Laboratory, Frederick National Laboratory for Cancer Research, Frederick, MD, USA., Kang E; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Malech HL; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA., Suffredini AF; Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD, USA., Noveck RJ; Duke Clinical Research Unit, Duke University School of Medicine, Durham, NC, USA., Dinubile MJ; BioAegis Therapeutics, Inc., North Brunswick, NJ, USA., Levinson SL; BioAegis Therapeutics, Inc., North Brunswick, NJ, USA., Stossel TP; BioAegis Therapeutics, Inc., North Brunswick, NJ, USA., Gallin JI; Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, USA. jig@nih.gov.
المصدر: Inflammation [Inflammation] 2021 Feb; Vol. 44 (1), pp. 270-277. Date of Electronic Publication: 2020 Sep 04.
نوع المنشور: Journal Article
اللغة: English
بيانات الدورية: Publisher: Kluwer Academic/Plenum Publishers Country of Publication: United States NLM ID: 7600105 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1573-2576 (Electronic) Linking ISSN: 03603997 NLM ISO Abbreviation: Inflammation Subsets: MEDLINE
أسماء مطبوعة: Publication: 1999- : New York, NY : Kluwer Academic/Plenum Publishers
Original Publication: New York, Plenum Press.
مواضيع طبية MeSH: Gelsolin/*blood , Granulomatous Disease, Chronic/*blood , Granulomatous Disease, Chronic/*diagnosis, Adolescent ; Adult ; Biomarkers/blood ; Bone Marrow Transplantation/methods ; Cohort Studies ; Endotoxins/toxicity ; Female ; Fever/blood ; Fever/chemically induced ; Fever/therapy ; Humans ; Male ; Middle Aged ; Young Adult
مستخلص: Plasma gelsolin (pGSN) is the secreted isoform of an intracellular actin remodeling protein found in high concentrations in human plasma. Clinical studies demonstrate reduced pGSN concentrations in several disease states, including severe trauma, burns, and sepsis. Markedly decreased pGSN concentrations in these conditions precede and predict adverse clinical outcomes. In this study, we measured pGSN in patients with chronic granulomatous disease (CGD), a primary immunodeficiency characterized by recurrent infections and dysregulated inflammation. pGSN was quantified using a sandwich ELISA in plasma from healthy volunteers, clinically stable CGD patients, and X-linked CGD carriers and in sera from 12 CGD patients undergoing bone marrow transplantation. pGSN was also quantified in healthy volunteers challenged with intravenous endotoxin. pGSN concentrations were lower in CGD patients without active infection or systemic inflammation compared with healthy control subjects. In CGD patients undergoing bone marrow transplantation, pGSN concentrations increased significantly following successful transplant. X-linked carriers of CGD had normal pGSN. Despite reduction of pGSN in CGD patients, we did not detect significant changes in pGSN over 24 h following challenge of healthy volunteers with intravenous endotoxin (4 ng/kg) that elicited a febrile response. We describe, for the first time, significantly lower pGSN in clinically stable patients with CGD compared with age- and sex-matched healthy volunteers. Low pGSN levels in CGD patients significantly increased following bone marrow transplantation. X-linked carriers of CGD had normal pGSN. In healthy volunteers challenged with intravenous endotoxin, pGSN is not an acute phase reactant.
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معلومات مُعتمدة: Z99 AI999999 United States ImNIH Intramural NIH HHS; ZIA AI001151 United States ImNIH Intramural NIH HHS; HHSN261200800001C United States CA NCI NIH HHS; ZIA AI000155 United States ImNIH Intramural NIH HHS; HHSN261200800001E United States CA NCI NIH HHS
فهرسة مساهمة: Keywords: CGD; endotoxin; gelsolin; inflammation
المشرفين على المادة: 0 (Biomarkers)
0 (Endotoxins)
0 (Gelsolin)
تواريخ الأحداث: Date Created: 20200905 Date Completed: 20211007 Latest Revision: 20240615
رمز التحديث: 20240615
مُعرف محوري في PubMed: PMC10825630
DOI: 10.1007/s10753-020-01330-w
PMID: 32886268
قاعدة البيانات: MEDLINE
الوصف
تدمد:1573-2576
DOI:10.1007/s10753-020-01330-w