title: Relation of streptococcal pyrogenic exotoxin C as a causative superantigen for Kawasaki disease.
abstract: We previously reported that the frequency of TCRBV2 and TCRBV6S5-bearing T-cells was high in patients in the acute phase of Kawasaki disease (KD) and that streptococcal pyrogenic exotoxin C (SPE-C) was a potent stimulator of these TCRBV-bearing T-cells. To further elucidate the pathogenesis of KD, we examined the T-cell receptor (TCR) repertoire, human leukocyte antigen (HLA)-DRB1 genotype, and antibody responses to recombinant(r) SPE-C in patients with KD. We also performed in vitro stimulation with rSPE-A and rSPE-C of peripheral blood mononuclear cells from healthy donors and characterized the reacting T-cells. The percentage of T-cells bearing TCRBV2 and TCRBV6S5 was high in patients in the acute stage of KD. rSPE-C stimulation of PBMC from healthy donors induced expansion of TCRBV2 and TCRBV6S5-bearing T-cells. Furthermore, serum levels of anti-SPEC antibodies, which did not display antimitogenic activity, were higher in patients with acute KD than in age-matched controls. The frequencies of the DRB1*04051, 0406, and 0901 were high, whereas that of the DRB1*1101 was low among patients with KD as compared with the healthy adults.
authors.affiliation: Shionogi Discovery Research Laboratories, Shionogi & Co, Ltd, Osaka-city, Osaka 533, Japan. takeshi.yoshioka@shionogi.co.jp
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yoshioka
authors.forename: Takaji
authors.initials: T
authors.lastname: Matsutani
authors.forename: Tomoko
authors.initials: T
authors.lastname: Toyosaki-Maeda
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Suzuki
authors.forename: Sigeru
authors.initials: S
authors.lastname: Uemura
authors.forename: Ryuji
authors.initials: R
authors.lastname: Suzuki
authors.forename: Michio
authors.initials: M
authors.lastname: Koike
authors.forename: Yorio
authors.initials: Y
authors.lastname: Hinuma
mesh_terms: D000907:Antibodies, Bacterial / Q000097:blood; D001426:Bacterial Proteins; D002675:Child, Preschool; D005098:Exotoxins / Q000032:analysis* / Q000276:immunology; D005260:Female; D005787:Gene Frequency; D005838:Genotype; D006684:HLA-DR Antigens / Q000235:genetics; D059811:HLA-DRB1 Chains; D006801:Humans; D007223:Infant; D008297:Male; D008565:Membrane Proteins; D009080:Mucocutaneous Lymph Node Syndrome / Q000235:genetics / Q000276:immunology* / Q000382:microbiology*; D013290:Streptococcal Infections / Q000150:complications* / Q000235:genetics / Q000276:immunology*; D018089:Superantigens / Q000032:analysis / Q000276:immunology; D013601:T-Lymphocytes / Q000382:microbiology
chemical_list: D000907:Antibodies, Bacterial; D001426:Bacterial Proteins; D005098:Exotoxins; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; D008565:Membrane Proteins; C117980:SpeA protein, Streptococcus pyogenes; D018089:Superantigens; C027951:erythrogenic toxin