PMID- 32278322
OWN - NLM
STAT- MEDLINE
DCOM- 20201013
LR  - 20201013
IS  - 1365-2249 (Electronic)
IS  - 0009-9104 (Print)
IS  - 0009-9104 (Linking)
VI  - 201
IP  - 1
DP  - 2020 Jul
TI  - Monocyte-derived dendritic cells display a highly activated phenotype and altered 
      function in patients with familial Mediterranean fever.
PG  - 1-11
LID - 10.1111/cei.13439 [doi]
AB  - Dendritic cells (DCs) are sentinels of the immune system that bridge innate and 
      adaptive immunity. By capturing antigens in peripheral tissue, processing and 
      presenting them with concurrent expression of co-stimulatory molecules and 
      cytokine secretion they control and modulate immune reactions. Through pattern 
      recognition receptors, DCs sense molecules that are associated with infection or 
      tissue damage, frequently resulting in the formation of inflammasomes upon 
      intracellular stimulation. The inherited autoinflammatory familial Mediterranean 
      fever (FMF) is associated with deregulated activity of the pyrin inflammasome 
      leading to acute inflammatory episodes. However, differentiation and function of 
      DCs in this disease are as yet unclear. Therefore, we first determined DC 
      subpopulation frequency in peripheral blood of a cohort of FMF patients. Joint 
      evaluation without classification according to specific patient characteristics, 
      such as mutational status, did not disclose significant differences compared to 
      healthy controls. For the further examination of phenotype and function, we used 
      immature and mature monocyte-derived DCs (imMo-DCs, mMo-DCs) that were generated 
      in vitro from FMF patients. Immunophenotypical analysis of imMo-DCs revealed a 
      significantly elevated expression of CD83, CD86 and human leukocyte antigen 
      D-related (HLA-DR) as well as a significant down-regulation of CD206, CD209 and 
      glycoprotein NMB (GPNMB) in our FMF patient group. Furthermore, FMF imMo-DCs 
      presented a significantly higher capacity to migrate and to stimulate the 
      proliferation of unmatched allogeneic T cells. Finally, the transition towards a 
      more mature, and therefore activated, phenotype was additionally reinforced by 
      the fact that peripheral blood DC populations in FMF patients exhibited 
      significantly increased expression of the co-stimulatory molecule CD86.
CI  - (c) 2020 The Authors. Clinical & Experimental Immunology published by John Wiley & 
      Sons Ltd on behalf of British Society for Immunology.
FAU - Funk, T
AU  - Funk T
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Fuchs, A R
AU  - Fuchs AR
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Altdorfer, V S
AU  - Altdorfer VS
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Klein, R
AU  - Klein R
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Autenrieth, S E
AU  - Autenrieth SE
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Muller, M R
AU  - Muller MR
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Salih, H R
AU  - Salih HR
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
AD  - 2Clinical Collaboration Unit (CCU) Translational Immunology, German Cancer 
      Consortium (DKTK) and German Cancer Research Center (DKFZ), Partner Site 
      Tubingen, Tubingen, Germany.
FAU - Henes, J
AU  - Henes J
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Grunebach, F
AU  - Grunebach F
AUID- ORCID: 0000-0002-9960-9865
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
FAU - Dorfel, D
AU  - Dorfel D
AD  - Department of Medical Oncology, Haematology, Clinical Immunology and 
      Rheumatology, University Hospital Tubingen, Tubingen, Germany.
AD  - 2Clinical Collaboration Unit (CCU) Translational Immunology, German Cancer 
      Consortium (DKTK) and German Cancer Research Center (DKFZ), Partner Site 
      Tubingen, Tubingen, Germany.
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20200427
PL  - England
TA  - Clin Exp Immunol
JT  - Clinical and experimental immunology
JID - 0057202
RN  - 0 (Antigens, Differentiation)
SB  - IM
MH  - Adult
MH  - Antigens, Differentiation/immunology
MH  - Cell Movement/*immunology
MH  - Dendritic Cells/*immunology/pathology
MH  - Familial Mediterranean Fever/*immunology/pathology
MH  - Humans
MH  - Male
MH  - Monocytes/*immunology/pathology
PMC - PMC7290084
OTO - NOTNLM
OT  - dendritic cells
OT  - familial Mediterranean fever
OT  - inflammation
COIS- The authors declare no competing financial interests.
EDAT- 2020/04/12 06:00
MHDA- 2020/10/21 06:00
PMCR- 2020/04/27
CRDT- 2020/04/12 06:00
PHST- 2019/07/16 00:00 [received]
PHST- 2020/03/31 00:00 [revised]
PHST- 2020/04/01 00:00 [accepted]
PHST- 2020/04/12 06:00 [pubmed]
PHST- 2020/10/21 06:00 [medline]
PHST- 2020/04/12 06:00 [entrez]
PHST- 2020/04/27 00:00 [pmc-release]
AID - CEI13439 [pii]
AID - 10.1111/cei.13439 [doi]
PST - ppublish
SO  - Clin Exp Immunol. 2020 Jul;201(1):1-11. doi: 10.1111/cei.13439. Epub 2020 Apr 27.