title: Expression of the costimulatory molecule BB-1 and its receptors in patients with scleroderma-polymyositis overlap syndrome.
abstract: We investigated expression of costimulatory molecules BB-1, B7-1 (CD80), B7-2 (CD86), and their counter-receptors CD28 and CTLA-4 (CD152) in muscle biopsy specimens of patients with scleroderma-polymyositis overlap syndrome (SSc-PM), primary polymyositis (PM), and other related diseases to examine whether the muscle fibers in patients with SSc-PM behave as antigen-presenting cells (APCs). The major histocompatibility (MHC) class II-positive muscle fibers of SSc-PM patients reacted with monoclonal antibodies (mAb) against BB-1 but not against B7-1 or B7-2. The CD4+ T cells expressed the counter-receptors CD28 and CTLA-4, and bound with the BB-1-positive muscle fibers in cell-to-cell contact. Our findings show that muscle fibers in patients with SSc-PM function as "professional" APCs in a way distinct from muscle fibers in patients with primary PM.
authors.affiliation: Department of Neurology, Nara Medical University, 840 Shijo-cho, Kashihara, Nara 634-8521, Japan. kemurata@naramed-u.ac.jp
authors.forename: Ken-ya
authors.initials: KY
authors.lastname: Murata
authors.forename: Kazuma
authors.initials: K
authors.lastname: Sugie
authors.forename: Miwa
authors.initials: M
authors.lastname: Takamure
authors.forename: Satoshi
authors.initials: S
authors.lastname: Ueno
mesh_terms: D000069594:Abatacept; D000328:Adult; D000368:Aged; D015703:Antigens, CD; D000943:Antigens, Differentiation / Q000235:genetics / Q000378:metabolism; D000944:Antigens, Differentiation, B-Lymphocyte / Q000235:genetics / Q000378:metabolism; D018122:B7-1 Antigen / Q000235:genetics / Q000378:metabolism*; D001426:Bacterial Proteins / Q000378:metabolism; D001706:Biopsy; D018106:CD28 Antigens / Q000235:genetics / Q000378:metabolism*; D015704:CD4 Antigens / Q000235:genetics / Q000276:immunology; D016827:CD8 Antigens / Q000235:genetics / Q000378:metabolism; D060908:CTLA-4 Antigen; D005260:Female; D000949:Histocompatibility Antigens Class II / Q000235:genetics / Q000378:metabolism; D006801:Humans; D018796:Immunoconjugates; D007150:Immunohistochemistry; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications / Q000235:genetics / Q000378:metabolism*; D012333:RNA, Messenger / Q000378:metabolism; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012595:Scleroderma, Systemic / Q000150:complications / Q000235:genetics / Q000378:metabolism*
chemical_list: D015703:Antigens, CD; D000943:Antigens, Differentiation; D000944:Antigens, Differentiation, B-Lymphocyte; D018122:B7-1 Antigen; D001426:Bacterial Proteins; D018106:CD28 Antigens; D015704:CD4 Antigens; D016827:CD8 Antigens; D060908:CTLA-4 Antigen; C556706:CTLA4 protein, human; D000949:Histocompatibility Antigens Class II; D018796:Immunoconjugates; C095564:Map protein, Staphylococcus aureus; D012333:RNA, Messenger; C045084:invariant chain; D000069594:Abatacept