|
|
 Previous Article | Table of Contents | Next Article 
Blood, Vol. 96 No. 2 (July 15), 2000:
pp. 727-731
Expression of the familial Mediterranean fever gene and activity
of the C5a inhibitor in human primary fibroblast cultures
Yaacov Matzner,
Suzan Abedat,
Eli Shapiro,
Shlomit Eisenberg,
Ariela Bar-Gil-Shitrit,
Polina Stepensky,
Sima Calco,
Yehudit Azar, and
Simcha Urieli-Shoval
From the Hematology Unit, Hadassah University Hospital, Jerusalem,
Israel.
Familial Mediterranean fever (FMF) is an inherited disease whose
manifestations are acute but reversible attacks of sterile inflammation
affecting synovial and serosal spaces. The FMF gene (MEFV) was recently cloned, and it codes for a protein
(pyrin/marenostrin) homologous to known nuclear factors. We previously
reported the deficient activity of a C5a/interleukin (IL)-8 inhibitor,
a physiologic regulator of inflammatory processes, in FMF serosal and
synovial fluids. We now describe the concomitant expression of
MEFV and C5a/IL-8-inhibitor activity in primary
cultures of human fibroblasts. Fibroblasts grown from synovial and
peritoneal tissues displayed C5a/IL-8-inhibitor activity that could be
further induced with phorbol myristate acetate (PMA) and IL-1 . Very
low levels of chemotactic inhibitor were evident in skin fibroblast
cultures or in peritoneal and skin fibroblasts obtained from FMF
patients. MEFV was expressed in peritoneal and skin fibroblasts
at a lower level than in neutrophils and could be further induced by
PMA and IL-1. In the FMF cultures, the MEFV transcript
carried the M694V mutation, consistent with the genetic defect found in
patients with this disease. MEFV was also expressed in other
cell lines that do not produce C5a/IL-8 inhibitor. These findings
suggest that human primary fibroblast cultures express MEFV and
produce C5a/IL-8-inhibitor activity. The interrelationship between
pyrin, the MEFV product, and the C5a/IL-8 inhibitor requires
further investigation.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
S. Farasat, I. Aksentijevich, and J. R. Toro
Autoinflammatory Diseases: Clinical and Genetic Advances
Arch Dermatol,
March 1, 2008;
144(3):
392 - 402.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Simon and J. W. M. van der Meer
Pathogenesis of familial periodic fever syndromes or hereditary autoinflammatory syndromes
Am J Physiol Regulatory Integrative Comp Physiol,
January 1, 2007;
292(1):
R86 - R98.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
J. J. Chae, G. Wood, S. L. Masters, K. Richard, G. Park, B. J. Smith, and D. L. Kastner
The B30.2 domain of pyrin, the familial Mediterranean fever protein, interacts directly with caspase-1 to modulate IL-1beta production
PNAS,
June 27, 2006;
103(26):
9982 - 9987.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E Rabinovich, A Livneh, P Langevitz, N Brezniak, E Shinar, M Pras, and Y Shinar
Severe disease in patients with rheumatoid arthritis carrying a mutation in the Mediterranean fever gene
Ann Rheum Dis,
July 1, 2005;
64(7):
1009 - 1014.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S Rosengren, H M Hoffman, W Bugbee, and D L Boyle
Expression and regulation of cryopyrin and related proteins in rheumatoid arthritis synovium
Ann Rheum Dis,
May 1, 2005;
64(5):
708 - 714.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. C. Braun, R. Y. Reins, T.-b. Li, T. J. Hollmann, R. Dutta, W. A. Rick, B.-B. Teng, and B. Ke
Renal Expression of the C3a Receptor and Functional Responses of Primary Human Proximal Tubular Epithelial Cells
J. Immunol.,
September 15, 2004;
173(6):
4190 - 4196.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K Konstantopoulos, A Kanta, C Deltas, V Atamian, D Mavrogianni, A G Tzioufas, I Kollainis, K Ritis, and H M Moutsopoulos
Familial Mediterranean fever associated pyrin mutations in Greece
Ann Rheum Dis,
May 1, 2003;
62(5):
479 - 481.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. A. Wise, J. D. Gillum, C. E. Seidman, N. M. Lindor, R. Veile, S. Bashiardes, and M. Lovett
Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder
Hum. Mol. Genet.,
April 15, 2002;
11(8):
961 - 969.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Richards, P. Schaner, A. Diaz, J. Stuckey, E. Shelden, A. Wadhwa, and D. L. Gumucio
Interaction between Pyrin and the Apoptotic Speck Protein (ASC) Modulates ASC-induced Apoptosis
J. Biol. Chem.,
October 12, 2001;
276(42):
39320 - 39329.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Mansfield, J. J. Chae, H. D. Komarow, T. M. Brotz, D. M. Frucht, I. Aksentijevich, and D. L. Kastner
The familial Mediterranean fever protein, pyrin, associates with microtubules and colocalizes with actin filaments
Blood,
August 1, 2001;
98(3):
851 - 859.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. J. DiMartino, A. B. Shah, G. Trujillo, and R. R. Kew
Elastase Controls the Binding of the Vitamin D-Binding Protein (Gc-Globulin) to Neutrophils: A Potential Role in the Regulation of C5a Co-Chemotactic Activity
J. Immunol.,
February 15, 2001;
166(4):
2688 - 2694.
[Abstract]
[Full Text]
[PDF]
|
|
|
| |
