Prohibitin Is Involved in Patients with IgG4 Related Disease.
OBJECTIVE
IgG4-related disease (IgG4-RD) is a chronic systemic disease involved in many organs and tissues. As only limited autoantigens have been found since the beginning of this century, the aim of this study was to reveal new candidate autoantigens of IgG4-RD.


METHODS
Multiple cell lines including HT-29, EA.hy926, HEK 293 and HepG2 were used to test the binding ability of circulating autoantibodies from IgG4-RD sera. The amino-acid sequence was then analyzed by matrix-assisted laser desorption/ionization time-of-flight tandem (MALDI-TOF/TOF) mass spectrometry. After the cloning and expression of recombinant putative autoantigen in a bacterial expression system, the corresponding immuno assay was set up and utilized to observe the prevalence of serum autoantibodies in a large set of confirmed clinical samples.


RESULTS
One positive autoantigen was identified as prohibitin. ELISA analysis showed that a majority of patients with IgG4-RD have antibodies against prohibitin. Anti-prohibitin antibodies were present in the sera of patients with definite autoimmune pancreatitis (25/34; 73.5%), Mikulicz's disease (8/15; 53.3%), retroperitoneal fibrosis (6/11; 54.5%), other probable IgG4-RD (26/29; 89.7%) and Sjögren's syndrome (4/30; 13.3%) but not in apparently healthy donors (1/70; 1.4%).


CONCLUSIONS
An association between prohibitin and patients with some IgG4-RD was observed, although the results were quite heterogeneous among different individuals within autoimmune pancreatitis, Mikulicz's disease and retroperitoneal fibrosis.
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Hongwu
H
Du
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Lili
L
Shi
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Peng
P
Chen
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Weikang
W
Yang
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Yiping
Y
Xun
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Chunhe
C
Yang
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Lanqing
L
Zhao
112 Lab, School of Chemistry and Biological Engineering, University of Science and Technology Beijing, Beijing, China.
Yabin
Y
Zhou
ImmunoHunt Corporation, Beijing, China.
Guangyu
G
Chen
D000293:Adolescent; D000328:Adult; D000941:Antigens / Q000378:metabolism; D001327:Autoimmune Diseases / Q000276:immunology*; D015153:Blotting, Western; D002460:Cell Line; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D047468:Immunoprecipitation; D008297:Male; D008875:Middle Aged; D000091402:Prohibitins; D011994:Recombinant Proteins / Q000302:isolation & purification / Q000378:metabolism; D012097:Repressor Proteins / Q000276:immunology / Q000378:metabolism*; D015203:Reproducibility of Results; D055815:Young Adult
D000941:Antigens; D007074:Immunoglobulin G; D000091402:Prohibitins; D011994:Recombinant Proteins; D012097:Repressor Proteins