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. 2001 Aug;60(8):777–780. doi: 10.1136/ard.60.8.777

Specific glycosylation of α1-acid glycoprotein characterises patients with familial Mediterranean fever and obligatory carriers of MEFV

D Poland 1, J Drenth 1, E Rabinovitz 1, A Livneh 1, J Bijzet 1, Hof B van het 1, W van Dijk 1
PMCID: PMC1753799  PMID: 11454642

Abstract

BACKGROUND—Familial Mediterranean fever (FMF) is a periodic febrile disorder, characterised by fever and serositis. The acute phase response during attacks of FMF results from the release of cytokines, which in turn induce increased expression and changed glycosylation of acute phase proteins. A recent study indicated that attacks in FMF are accompanied by a rise of plasma concentrations of serum amyloid A (SAA) and C reactive protein (CRP), which remain significantly raised during remission relative to healthy controls. Another study suggested that obligatory heterozygotes also display an inflammatory acute phase response.
OBJECTIVE—To determine the state of inflammation in homozygotic and heterozygotic MEFV genotypes.
METHODS—CRP and SAA were studied by enzyme linked immunosorbent assay (ELISA). The glycosylation of the acute phase protein, α1-acid glycoprotein (AGP), was visualised with crossed affinoimmunoelectrophoresis with concanavalin A as diantennary glycan-specific component and Aleuria aurantia lectin as fucose-specific affinity component.
RESULTS—FMF attacks were associated with an increase (p<0.05) in the serum inflammation parameters CRP, SAA, and AGP. The glycosylation of AGP showed an increase (p<0.05) in fucosylated AGP glycoforms, whereas the branching of the glycans remained unaffected. The glycosylation of AGP in the MEFV carrier group, compared with that in a healthy control group, was characterised by a significant increase (p<0.05) in branching of the glycans, whereas the fucosylation remained unaffected.
CONCLUSION—The findings suggest an FMF-specific release of cytokines, resulting in a different glycosylation of AGP between a homozygotic and heterozygotic MEFV genotype.



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Figure 1  .

Figure 1  

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Crossed affinoimmunoelectrophoresis patterns of α1 acid glycoprotein (AGP) with (A-D) concanavalin A (Con A) and (E-H) Aleuria aurantia lectin (AAL) in serum of a representative control (A, E), MEFV carrier (B, F), patient with familial Mediterranean fever (FMF) during attack (C, G), and a patient with FMF during remission (D, H). The first dimension gel, containing the lectin, was electrophoresed from right to left, the second dimension gel, containing the anti-AGP antiserum, from bottom to top; see "Material and methods" for experimental details. C0 and A0 = AGP glycoforms that are non-reactive with Con A and AAL, respectively; Cw and Aw = AGP glycoforms that are weakly reactive with Con A and AAL, respectively; Cs and As = AGP glycoforms that are strongly reactive with Con A and AAL, respectively.

Selected References

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