Entry Name Systemic lupus erythematosus - Pteropus vampyrus (large flying fox)
Description Systemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterised by the production of IgG autoantibodies that are specific for self-antigens, such as DNA, nuclear proteins and certain cytoplasmic components, in association with a diverse array of clinical manifestations. The primary pathological findings in patients with SLE are those of inflammation, vasculitis, immune complex deposition, and vasculopathy. Immune complexes comprising autoantibody and self-antigen is deposited particulary in the renal glomeruli and mediate a systemic inflammatory response by activating complement or via Fc{gamma}R-mediated neutrophil and macrophage activation. Activation of complement (C5) leads to injury both through formation of the membrane attack complex (C5b-9) or by generation of the anaphylatoxin and cell activator C5a. Neutrophils and macrophages cause tissue injury by the release of oxidants and proteases.
Class Human Diseases; Immune diseaseBRITE hierarchy
Pathway map Ortholog table Organism Pteropus vampyrus (large flying fox) [GN:
pvp ]
Gene 105298226 SNRPB; small nuclear ribonucleoprotein-associated proteins B and B' [KO:K11086 ]
105292281 SNRPD1; small nuclear ribonucleoprotein Sm D1 isoform X1 [KO:K11087 ]
105305274 HLA class II histocompatibility antigen, DR alpha chain [KO:K06752 ]
105305333 DLA class II histocompatibility antigen, DR-1 beta chain [KO:K06752 ]
105301039 LOW QUALITY PROTEIN: HLA class II histocompatibility antigen, DP beta 1 chain [KO:K06752 ]
105301004 LOW QUALITY PROTEIN: DLA class II histocompatibility antigen, DR-1 beta chain-like [KO:K06752 ]
105300960 HLA class II histocompatibility antigen, DQ alpha 2 chain-like [KO:K06752 ]
105300961 HLA class II histocompatibility antigen, DQ beta 1 chain isoform X1 [KO:K06752 ]
105300962 HLA class II histocompatibility antigen, DQ alpha 2 chain-like [KO:K06752 ]
105300964 HLA class II histocompatibility antigen, DO beta chain [KO:K06752 ]
105300969 HLA class II histocompatibility antigen, DM beta chain [KO:K06752 ]
105300971 HLA class II histocompatibility antigen, DM alpha chain isoform X3 [KO:K06752 ]
105300973 HLA class II histocompatibility antigen, DO alpha chain isoform X1 [KO:K06752 ]
105309227 HLA class II histocompatibility antigen, DR beta 5 chain-like [KO:K06752 ]
105310204 SLA class II histocompatibility antigen, DQ haplotype D alpha chain-like [KO:K06752 ]
105310338 HLA class II histocompatibility antigen, DQ beta 1 chain-like [KO:K06752 ]
105298423 CD28; T-cell-specific surface glycoprotein CD28 isoform X1 [KO:K06470 ]
105298191 CD40; tumor necrosis factor receptor superfamily member 5 isoform X1 [KO:K03160 ]
105309973 high affinity immunoglobulin gamma Fc receptor I-like isoform X1 [KO:K06498 ]
105303404 low affinity immunoglobulin gamma Fc region receptor III-B-like isoform X1 [KO:K06463 ]
Compound Reference Authors Rahman A, Isenberg DA.
Title Systemic lupus erythematosus.
Journal Reference Authors Mok CC, Lau CS.
Title Pathogenesis of systemic lupus erythematosus.
Journal Reference Authors Clatworthy MR, Smith KG.
Title B cells in glomerulonephritis: focus on lupus nephritis.
Journal Reference Authors Couser WG.
Title New insights into mechanisms of immune glomerular injury.
Journal West J Med 160:440-6 (1994)
Reference Authors Cook HT, Botto M.
Title Mechanisms of Disease: the complement system and the pathogenesis of systemic lupus erythematosus.
Journal Reference Authors Carroll MC.
Title A protective role for innate immunity in systemic lupus erythematosus.
Journal Reference Authors Sturfelt G, Truedsson L.
Title Complement and its breakdown products in SLE.
Journal Reference Authors Lu L, Kaliyaperumal A, Boumpas DT, Datta SK.
Title Major peptide autoepitopes for nucleosome-specific T cells of human lupus.
Journal Reference Authors Zieve GW, Khusial PR.
Title The anti-Sm immune response in autoimmunity and cell biology.
Journal Reference Authors Scofield RH, Farris AD, Horsfall AC, Harley JB.
Title Fine specificity of the autoimmune response to the Ro/SSA and La/SSB ribonucleoproteins.
Journal Reference Authors Hoffman RW.
Title T cells in the pathogenesis of systemic lupus erythematosus.
Journal Related pvp04060 Cytokine-cytokine receptor interaction
pvp04610 Complement and coagulation cascades
pvp04612 Antigen processing and presentation
pvp04660 T cell receptor signaling pathway
pvp04662 B cell receptor signaling pathway
pvp04670 Leukocyte transendothelial migration
KO pathway LinkDB All DBs
